Search

Introduction: Cerebrospinal fluid (CSF) fistulas are a rare phenomenon, that can lead to life-threatening complications if left untreated. Presenting as rhinorrhea or otorrhea, they can be difficult to diagnose due to admixture of other bodily fluids. Typically, CSF fistulas develop after trauma, but in rare instances, they can be diagnosed in patients with a neoplastic lesion., Objective: To discuss several steps in diagnosing CSF fistulas., Patient: A fifty-year-old female with an intra-osseous temporal bone meningioma., Interventions: For diagnosing CSF admixture in fluids, two tests are looked into: beta-2 transferrin (β2T) and beta-trace protein (βTP) testing., Conclusion: Testing for βTP is a highly sensitive, quick and non-invasive method to assess CSF admixture in middle ear effusion. Because of its lower cost, faster results and easy sample collection, βTP testing has in our clinic replaced β2T testing. The current case illustrates a rare etiology of a CSF fistula, where β2T testing presumably showed false-negative results and βTP testing showed true-positive results., Competing Interests: The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (© 2024 The Authors. Published by Elsevier Ltd.)

Objective: This systematic review aims to describe the impact of otologic surgery as a treatment for chronic otitis media (COM) on the Health-Related Quality of Life (HRQoL) of adult patients., Methods: A literature search was performed in PubMed, Scopus, Embase, and Web of Science until May 2023. Prospective studies including adult patients with COM (cholesteatoma) who underwent canal wall up mastoidectomy, canal wall down mastoidectomy, or tympanoplasty without mastoidectomy, with pre- and postoperative HRQoL measurements, were considered eligible. Questionnaire validation studies were excluded. The risk of bias and study quality were evaluated with a Quality Assessment Tool (for before-after studies with no control group). To assess the change in HRQoL, pre- and postoperative HRQoL values and absolute changes were extracted, synthesized, and presented in tables. Standardized mean differences (SMD) were calculated to enhance comparisons., Results: Of the 720 studies identified, 16 met the inclusion criteria of this review. Different questionnaires were used throughout the studies. The CES and COMOT-15 were used in five studies and the ZCMEI-21 and COMQ-12 in three studies. All studies indicated statistically significant improvement in HRQoL from pre- to postoperative, measured with disease-specific HRQoL questionnaires. General HRQoL questionnaires did not show significant improvement. Calculated SMDs ranged from 0.24 to 6.99., Discussion and Conclusion: Included studies had low ( n = 10) to high ( n = 6) risk of bias and poor ( n = 4), fair ( n = 7) or good ( n = 5) study quality. Surgical treatment positively impacts the HRQoL of adult COM patients with and without cholesteatoma. However, the clinical relevance of the reported changes is unknown due to the lack of minimal clinically important differences (MCID) or cut-off values in each questionnaire. Therefore, further research regarding the MCIDs of each questionnaire is needed. Future research should also report preoperative chief symptoms and indications for surgery to improve individual patient counseling., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2023 Schouwenaar, Hellingman and Waterval.)

Objective: Vestibular schwannoma (VS) growth of ≥2 mm during serial MRI observation, irrespective of size, is the benchmark for treatment initiation in almost all centers. Although the probability of less optimal outcomes significantly increases in VS closer to the brainstem, early intervention does not improve long-term quality of life. Moving beyond the recommendation of definitive treatment for all VS after detected growth, we subclassified Koos 2 tumors based on extrameatal extension and relation to the brainstem. The aim of the current study was to evaluate the Koos 2 subclassification's validity and the inter-and intra-rater reliability of the entire Koos classification., Methods: Six experts, including neurosurgeons, otorhinolaryngologists and radiologists from two tertiary referral centers, classified 43 VS MRI scans. Validity of the Koos 2 subclassification was evaluated by the percentage agreement against the multidisciplinary skull base tumor board management advice. Inter- and intra-rater reliability were calculated using the intraclass correlation coefficient (ICC)., Results: Validity was almost perfect in Koos 2a VSs with a 100% agreement and 87.5% agreement for Koos 2b. Inter-rater reliability for all Koos grades was significantly excellent (ICC 0.91; 95%CI 0.866 to 0.944, p= <0.001). Five raters had an excellent intra-rater reliability (ICC > 0.90; p= <0.01) and one rater had a good intra-rater reliability (ICC 0.88; 95% CI 0.742 to 0.949)., Conclusions: Although multiple factors influence decision-making, the classification of Koos 2a and 2b with excellent inter- and intra-rater reliability, can aid in recommending treatment initiation, moving beyond detected tumor growth, aiming to optimize patient centered care., Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2023 The Authors. Published by Elsevier B.V. All rights reserved.)

Objectives: Otomastoiditis caused by the anaerobic Fusobacterium necrophorum (F. necrophorum) often induces severe complications, such as meningitis and sinus thrombosis. Early diagnosis is difficult, partly because little is known about specific early signs. Comprehensive research about clinically chosen antimicrobial therapy has not been done yet and prognostic information about otomastoiditis caused by F. necrophorum is scarce. More knowledge about this subject is required., Methods: In this retrospective cohort study, we included all cases of otomastoiditis caused by F. necrophorum treated in two university medical centres in the Netherlands during the past 10 years. Data was gathered from patient records and analysed using independent sample T-tests and Chi 2 -tests., Results: This study reveals that otomastoiditis caused by F. necrophorum potentially induces neurological sequelae. Thereby, 80% of all included patients (n = 16) needed readmission within six months due to recurrence or complications of otomastoiditis caused by F. necrophorum. Mean (range) of age, CRP and temperature were 4.5 years (0.9-29.3), 243 mg/L (113-423) and 40 °C (37-41). All patients were hospitalized and treated with antibiotics, mostly metronidazole (n = 13/16) and a β -lactam (n = 15/16). Additional treatment contained low molecular weight heparin (83%, n = 10/12), dexamethasone (78%, n = 7/9) and/or surgery (80%, n = 12/16, whereof 9/12 mastoidectomy)., Conclusions: Patients and/or their parents need to be informed about this potential unfortunate prognosis when otomastoiditis caused by F. necrophorum is diagnosed. To improve early diagnosis, otomastoiditis caused by F. necrophorum should be suspected and therefore immediately cultured when a) young children present with otomastoiditis, with b) high CRP values, and/or c) vomiting and decreased consciousness., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2022 The Authors. Published by Elsevier Ltd.. All rights reserved.)

Objectives: Necrotizing external otitis (NEO) is a rare infectious disease of the skull base. The purpose of this study was to determine whether clinical outcomes of NEO can be correlated to different infectious spread patterns., Methods: Retrospective chart review from 2010 to 2019 with NEO patients, who were divided into two cohorts: single spreading patterns (group A) or complex spreading patterns (group B) as diagnosed by CT. Clinical symptoms, diagnostic and treatment delay, course of disease, complications, and duration of antibiotic exposure were retrospectively collected from patient records., Results: 41 NEO patients were included, of which 27 patients belonged to group A (66%). The disease-related mortality rate was 12.2% among the entire cohort, no differences were found between group A and B. Higher rates of N.VII (42.9% vs 14.8% P = 0.047) and N. IX palsies were found in group B compared to group A (28.6% vs 3.7%, P = 0.039). The median duration of antibiotic use was significantly different for a complex spreading pattern, clinical recovery and hospitalizations. Complications were associated with higher diagnostic delay and with a complex spread pattern. The median duration of follow-up was 12.0 (IQR 6.0-19.5) months., Conclusion: NEO is a severe disease, with significant mortality and morbidity (cranial nerve palsies). The radiological spread pattern may assist in predicting clinical outcome. Furthermore, complex spread patterns are associated with higher rates of clinical nerve palsies (N. VII and N.IX), complications, surgery rates and longer duration of antibiotic use. Diagnostic delay was associated with mortality, complications and facial palsies., Level of Evidence: Level IV., Competing Interests: The authors declare no conflict of interests., (© 2022 PLA General Hospital Department of Otolaryngology Head and Neck Surgery. Production and hosting by Elsevier (Singapore) Pte Ltd.)

Background and Purpose: Necrotizing external otitis (NEO) is a serious complication of external otitis. NEO can be classified according to-anterior, medial, posterior, intracranial, and contralateral-extension patterns. Currently there is no consensus on the optimal imaging modality for the identification of disease extension. This study compares NEO extension patterns on MR and CT to evaluate diagnostic comparability., Methods: Patients who received a CT and MR within a 3-month interval were retrospectively examined. Involvement of subsites and subsequent spreading patterns were assessed on both modalities by a radiologist in training and by a senior head and neck radiologist. The prevalence of extension patterns on CT and MR were calculated and compared., Results: All 21 included NEO cases showed an anterior extension pattern on CT and MR. Contrary to MR, medial extension was not recognized on CT in two out of six patients, and intracranial extension in five out of eight patients. The posterior extension pattern was not recognized on MR. Overall, single anterior extension pattern (62%) is more prevalent than multiple extension patterns (38%)., Conclusion: All anterior NEO extension pattern were identified on CT as well as MR. However, the medial and intracranial spreading patterns as seen on MR could only be identified on CT in a small number of patients. The posterior spreading pattern can be overlooked on MR. Thus, CT and MR are complimentary for the initial diagnosis and work-up of NEO as to correctly delineate disease extent through the skull base., (© 2021. The Author(s).)

Objective: To describe a case of complete spontaneous regression of a middle ear melanoma., Patient: We present a case of a 68-year-old man with complaints of unilateral hearing loss and an ipsilateral facial nerve paresis. Radiological and histopathological examination revealed a cT4bN0M0 mucosal melanoma of the middle ear., Interventions: The patient underwent a subtotal petrosectomy and postoperative radiotherapy., Main Outcome Measure: Computed tomography (CT), magnetic resonance imaging (MRI), positron emission tomography/computed tomography with 2-[fluorine-18]-fluoro-2-deoxy-D-glucose (FDG-PET-CT), and histopathological examination., Results: After subtotal petrosectomy, histopathological examination of the resection specimen showed only fibrosis and a histiocytic and clonal T-cell infiltration, but no residual melanoma at the primary tumor site, consistent with spontaneous tumor regression. Follow-up MRI scanning 6 and 12 months after radiotherapy showed no signs of tumor recurrence., Conclusions: This case describes the concept of spontaneous regression of a mucosal melanoma of the middle ear. Spontaneous tumor regression at this location has not been described before., Competing Interests: The authors disclose no conflicts of interest., (Copyright © 2021 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of Otology & Neurotology, Inc.)

Objectives: Insertion of mold material into the middle ear is a complication of molding procedure for ear impression. These cases are referred to an ENT specialist. There is no standardized approach to this problem. Literature shows different clinical strategies. The aim of this study is to share our experience and to analyze the adverse outcome of different clinical approaches., Methods: A case series of six patients with molding material inside the middle ear after complicated molding procedure for swimming earplugs are described. Additionally, available literature was reviewed to analyze results of the clinical approach after iatrogenic molding procedures. Forty-nine ears were included., Results: In-office removal of the material is associated with a significant risk of adverse outcome if the eardrum cannot be examined. This also accounts for ossicular involvement., Conclusions: Temporal bone CT is advised in patients after complicated ear mold fitting if the tympanic membrane cannot be examined completely or the middle ear is involved. Blind removal should be avoided. Retroauricular transcanal tympanotomy or transmastoidal tympanotomy with facial recess approach is best practice in case the mold material has entered the middle ear. A clinical treatment algorithm is presented.

Background: Little is known about the growth rate of cholesteatoma in patients., Objective: Investigate the growth of residual cholesteatoma in subtotal petrosectomy based on volume measured in MRI scans., Materials and Methods: Retrospective case series in a Tertiary Medical Centre. Thirteen residual cholesteatomas were identified in 10 patients after subtotal petrosectomy for which a wait-and-scan policy was adopted. Volume of the residual cholesteatoma was calculated by manual segmentation as well as the 'box method'., Results: Mean growth rate was 27.9 mm3/month (SD 22.8), with a large individual variation ranging from 2.2 to 69.8 mm3/month. No complications were reported in 10 patients with a wait-and-scan policy for residual cholesteatoma in subtotal petrosectomy. The box method overestimates growth rate compared to the reference method manual segmentation and a linear increase of this systematic error was seen with increasing size of the cholesteatoma., Conclusions: Residual cholesteatoma growth rate shows a large individual variation. A wait-and-scan policy could be considered in case of a (small) residual in subtotal petrosectomy with ample room to grow before destroying any remaining structures. Furthermore, the clinically more applicable and less time-consuming box method can be used to accurately measure volumes of small cholesteatomasup to a volume of 500 mm 3 .

Hyperostosis Cranialis Interna (HCI) is a rare bone disorder characterized by progressive intracranial bone overgrowth at the skull. Here we identified by whole-exome sequencing a dominant mutation (L441R) in SLC39A14 (ZIP14). We show that L441R ZIP14 is no longer trafficked towards the plasma membrane and excessively accumulates intracellular zinc, resulting in hyper-activation of cAMP-CREB and NFAT signaling. Conditional knock-in mice overexpressing L438R Zip14 in osteoblasts have a severe skeletal phenotype marked by a drastic increase in cortical thickness due to an enhanced endosteal bone formation, resembling the underlying pathology in HCI patients. Remarkably, L438R Zip14 also generates an osteoporotic trabecular bone phenotype. The effects of osteoblastic overexpression of L438R Zip14 therefore mimic the disparate actions of estrogen on cortical and trabecular bone through osteoblasts. Collectively, we reveal ZIP14 as a novel regulator of bone homeostasis, and that manipulating ZIP14 might be a therapeutic strategy for bone diseases.

Objectives/hypothesis: To evaluate the perceptual effect of the altered shape of the osseous external auditory canal (OEAC) on sound quality., Study Design: Prospective study., Methods: Twenty subjects with normal hearing were presented with six simulated sound conditions representing the acoustic properties of six different ear canals (three normal ears and three cavities). The six different real ear unaided responses of these ear canals were used to filter Dutch sentences, resulting in six simulated sound conditions. A seventh unfiltered reference condition was used for comparison. Sound quality was evaluated using paired comparison ratings and a visual analog scale (VAS)., Results: Significant differences in sound quality were found between the normal and cavity conditions (all P < .001) using both the seven-point paired comparison rating and the VAS. No significant differences were found between the reference and normal conditions. Sound quality deteriorates when the OEAC is altered into a cavity., Conclusions: This proof of concept study shows that the altered acoustic quality of the OEAC after radical cavity surgery may lead to a clearly perceived deterioration in sound quality. Nevertheless, some questions remain about the extent to which these changes are affected by habituation and by other changes in middle ear anatomy and functionality., Level of Evidence: 4, (© 2015 The American Laryngological, Rhinological and Otological Society, Inc.)

In this review we provide a complete overview of the existing sclerosing bone dysplasias with craniofacial involvement. Clinical presentation, disease course, the craniofacial symptoms, genetic transmission pattern and pathophysiology are discussed. There is an emphasis on radiologic features with a large collection of CT and MRI images. In previous reviews the craniofacial area of the sclerosing bone dysplasias was underexposed. However, craniofacial symptoms are often the first symptoms to address a physician. The embryology of the skull and skull base is explained and illustrated for a better understanding of the affected areas., (Copyright © 2013 Elsevier Inc. All rights reserved.)

Background: In otosclerosis, CT of the temporal bone is used to confirm the diagnosis. Whereas CT is a static diagnostic tool displaying the demineralization caused by otosclerosis, imaging of bone metabolism by (18)F-fluoride PET may provide quantitative information. This could be useful for prognosis and stratification of patients. The aim of this study was to assess (18)F-fluoride activity in patients with otosclerosis and to evaluate its use as a complementary diagnostic tool., Methods: All patients with otosclerosis underwent a PET/CT scan. Audiometric data were collected. The severity of otosclerosis was assessed using a recognized radiological classification. The control group consisted of patients who had undergone (18)F-fluoride PET/CT scan for orthopedic purpose. Regions of interest were drawn on PET scans which corresponded to standardized anatomical sites as defined on CT, to measure bone metabolism using standardized uptake values (SUV(max) and SUV(mean))., Results: Group 1 consisted of 11 otosclerosis patients (16 eligible temporal bones) and group 2 consisted of 5 control patients (10 temporal bones). On PET scan, visual assessment of temporal bones with otosclerosis showed increased metabolic activity in the otic capsule in 11/16 cases. The SUV(max) in the entire otic capsule was significantly higher in otosclerosis patients compared to control subjects. Significant differences in SUV(mean) were found between otosclerosis and control subjects in the fenestral and saccule area. Moreover, metabolic activity in these regions significantly correlated with hearing loss and CT classification., Conclusions: (18)F-Fluoride PET scanning using SUV measurements has the potential to be a diagnostic tool in otosclerosis.

Objective: Hyperostosis cranialis interna (HCI) is an autosomal dominant sclerosing bone dysplasia affecting the skull base and the calvaria, characterized by cranial nerve deficits due to stenosis of neuroforamina. The aim of this study is to describe the value of several neurophysiological, audiometric and vestibular tests related to the clinical course of the disorder., Methods: Ten affected subjects and 13 unaffected family members were recruited and tested with visual evoked potentials, masseter reflex, blink reflex, pure tone and speech audiometry, stapedial reflexes, otoacoustic emissions, brainstem evoked response audiometry and electronystagmography., Results: Due to the symmetrical bilateral nature of this disease, the sensitivity of visual evoked potentials (VEPs), masseter reflex and blink reflex is decreased (25-37.5%), therefore reducing the value of single registration. Increased hearing thresholds and increased BERA latency times were found in 60-70%. The inter-peak latency I-V parameter in BERA has the ability to determine nerve encroachment reliably. 50% of the patients had vestibular abnormalities. No patient had disease-related absence of otoacoustic emissions, because the cochlea is not affected., Conclusion: In patients with HCI and similar craniofacial sclerosing bone dysplasias we advise monitoring of vestibulocochlear nerve function with tone and speech audiometry, BERA and vestibular tests. VEPs are important to monitor optic nerve function in combination with radiological and ophthalmologic examination. We do not advise the routine use of blink and masseter reflex., (Copyright © 2013 Elsevier B.V. All rights reserved.)

Hyperostosis cranialis interna (HCI) is a rare autosomal dominant disorder characterized by intracranial hyperostosis and osteosclerosis, which is confined to the skull, especially the calvarium and the skull base. The rest of the skeleton is not affected. Progressive bone overgrowth causes nerve entrapment that leads to recurrent facial nerve palsy, disturbance of the sense of smell, hearing and vision impairments, impairment of facial sensibility, and disturbance of balance due to vestibular areflexia. The treatment is symptomatic. Histomorphological investigations showed increased bone formation with a normal tissue structure. Biochemical parameters were normal. Until today the disease has been described in only three related Dutch families with common progenitors and which consist of 32 individuals over five generations. HCI was observed in 12 family members over four generations. Patients are mildly to severely affected. Besides HCI, several bone dysplasias with hyperostosis and sclerosis of the craniofacial bones are known. Examples are Van Buchem disease, sclerosteosis, craniometaphyseal dysplasia, and Camurati-Engelmann disease. However, in these cases the long bones are affected as well. Linkage analysis in a family with HCI resulted in the localization of the disease-causing gene to a region on chromosome 8p21 delineated by markers D8S282 and D8S382. Interesting candidate genes in this region are BMP1, LOXL2, and ADAM28. Sequence analysis of these genes did not reveal any putative mutations. This suggests that a gene not previously involved in a sclerosing bone dysplasia is responsible for the abnormal growth in the skull of these patients.

Background and Purpose: HCI is a unique autosomal-dominant sclerosing bone dysplasia affecting the skull base and the calvaria, characterized by cranial nerve deficits due to stenosis of neuroforamina, whereby the mandible is affected to a lesser extent. The aim of this study is to describe the specific radiologic characteristics and course of the disorder., Materials and Methods: CT scans of affected individuals within 1 family were analyzed and compared with scans of their unaffected family members and with an age- and sex-matched control group. Linear measurements were performed of the inner table, the medulla, and the outer table of different skull locations, and attenuation (density) measurements of the same regions were recorded. Neuroforamina widths were recorded as well., Results: There was significant thickening of the skull in the frontal, parietal, temporal, and occipital regions, which was mainly due to thickening of the inner table of the skull. The attenuation of the deposited hyperostotic bone was lower than normal cortical bone., Conclusions: HCI is the only genetic bone dysplasia known that is confined to the craniofacial area. The hyperostotic bone is less attenuated than normal cortical bone. The observed radiologic abnormalities explain the possible impairment of the olfactory, optic, trigeminal, facial, and vestibulocochlear nerves.

Purpose: (18)F-Fluoride PET/CT is a relatively undervalued diagnostic test to measure bone metabolism in bone diseases. Hyperostosis cranialis interna (HCI) is a (hereditary) bone disease characterised by endosteal hyperostosis and osteosclerosis of the skull and the skull base. Bone overgrowth causes entrapment and dysfunction of several cranial nerves. The aim of this study is to compare standardised uptake values (SUVs) at different sites in order to quantify bone metabolism in the affected anatomical regions in HCI patients., Methods: Nine affected family members, seven non-affected family members and nine non-HCI non-family members underwent (18)F-fluoride PET/CT scans. SUVs were systematically measured in the different regions of interest: frontal bone, sphenoid bone, petrous bone and clivus. Moreover, the average (18)F-fluoride uptake in the entire skull was measured by assessing the uptake in axial slides. Visual assessment of the PET scans of affected individuals was performed to discover the process of disturbed bone metabolism in HCI., Results: (18)F-Fluoride uptake is statistically significantly higher in the sphenoid bone and clivus regions of affected family members. Visual assessment of the scans of HCI patients is relevant in detecting disease severity and the pattern of disturbed bone metabolism throughout life., Conclusion: (18)F-Fluoride PET/CT is useful in quantifying the metabolic activity in HCI and provides information about the process of disturbed bone metabolism in this specific disorder. Limitations are a narrow window between normal and pathological activity and the influence of age. This study emphasises that (18)F-fluoride PET/CT may also be a promising diagnostic tool for other metabolic bone disorders, even those with an indolent course.

In recent years tomographic hybrid scanners have been quickly introduced in nuclear medicine: single-photon emission computed tomography (SPECT)-CT and positron emission tomography (PET)-CT.- Both SPECT-CT and PET-CT techniques provide a higher diagnostic accuracy than conventional (non-tomographic, non-hybrid) bone scintigraphy (bone scan).- Differences between 99mTc hydroxymethylene diphosphonate (HDP) SPECT-CT or 99mTc methylene diphosphonate (MDP) SPECT-CT and 18F-fluoride PET-CT bone scanning relate to image quality, technique, availability, quantification possibilities, radiation dosimetry and financial cost.- Indications for these techniques will especially lie in the field of more accurate detection of skeletal metastases than with bone scans, patients with unexplained musculoskeletal pain, the diagnostic stage after conventional X-ray and/or MRI, and quantification of bone metabolism.

Hyperostosis cranialis interna is a hereditary bone disorder that is characterized by endosteal hyperostosis and osteosclerosis of the calvaria and the skull base (OMIM 144755). The progressive bone overgrowth causes entrapment and dysfunction of cranial nerves I, II, V, VII, and VIII, its first symptoms often presenting during the second decade. This study analyzes the clinical course of 13 affected individuals of three related families (32 individuals). The disorder appears to have an autosomal-dominant transmission pattern. Facial and vestibulocochlear nerve dysfunction are most frequently reported. Surgical decompression of the accessible impaired cranial nerves is advised in the early symptomatic period or even in the presymptomatic period in high-risk individuals., ((c) 2010 Wiley-Liss, Inc.)

Unlabelled: Hyperostosis cranialis interna is an autosomal dominant disorder characterised by endosteal hyperostosis and osteosclerosis of the skull base and calvaria, leading to compression and dysfunction of cranial nerves I, II, VII and VIII., Case Report: We report the use of bilateral surgical decompression of the internal auditory canals to treat hyperostosis cranialis interna in an eight-year-old girl presenting with bilateral facial palsy due to hyperostosis cranialis interna., Intervention and Outcome: Using a middle fossa craniotomy approach, both internal auditory canals were unroofed and cranial nerves VII and VIII were decompressed, with a one-year interval between sides. The mimic function recovered. One year post-operatively, the right and left facial sides had been restored to House-Brackmann grades I and II, respectively., Conclusion: This is the first report of the use of surgical decompression of the internal auditory canal in a case of hyperostosis cranialis interna. Surgical decompression of the internal auditory canal is recommended therapeutically, but may also be performed prophylactically in younger patients with hyperostosis cranialis interna.