Search

Objective: To evaluate the literature and summarize cochlear implantation (CI) outcomes after intralabyrinthine schwannoma (ILS) excision and tumor observation with CI., Data Sources: OVID Medline, Embase, Web of Science; conception to 2024., Review Methods: A literature review was performed using subject headings, MeSH terms, and keywords. Abstracts and full texts were reviewed by two independent reviewers and adjudicated by a third. Inclusion criteria included studies with ILS and CI with reported audiologic outcomes. Subjects were analyzed into two groups, ILS resection with CI and in situ ILS with CI. Patients with NF2 were included. The main outcome of interest was CI audiometric performance level, with secondary outcomes of CI user status and open-set speech attainment., Results: There were 29 articles with a total of 93 patients who met inclusion criteria. The resection group had 17% low performers, 44% intermediate performers, and 38% high performers. The in situ group had 40% low performers, 32% intermediate performers, 27% high performers. The resection group had 69 patients with 97% maintaining user status and 92% with open-set speech recognition. The observation group had 24 patients, with 87% user rate and 86% achieving open-set speech recognition. There was a greater percentage of NF2 diagnosis seen in the in situ group., Conclusion: There is a paucity of literature on CI and ILS. Patients are managed with both resection of tumor and implantation in situ. Early data are limited, with improvement in hearing outcomes and high user rates in both populations., Level of Evidence: N/A Laryngoscope, 134:3910-3920, 2024., (© 2024 The American Laryngological, Rhinological and Otological Society, Inc.)

Objectives: The role of traditional nuclear medicine studies in the management of malignant otitis externa (MOE) is unclear and there are ongoing debates regarding their diagnostic value. The authors perform a systematic review and meta-analysis to assess the sensitivity and specificity of traditional nuclear medicine studies in the diagnosis of MOE., Methods: In accordance with PRISMA guidelines, a query of the Medline, Embase, Web of Science, and Cochrane databases was undertaken. The primary outcomes of interest were the sensitivity and specificity of traditional nuclear medicine studies to detect MOE., Results: Of the initial 1317 hits from the four databases, 20 articles with a combined 608 patients were ultimately included in the review. The pooled sensitivities for Technetium-99 and Gallium-67 were 85.1% (95% CI, 72.0-98.1%) and 71.2% (95% CI, 55.1-87.3%) respectively. The available evidence suggested poor specificity of these modalities, but was insufficient for meta-analysis. Neither modality was shown to be effective in the assessment of disease resolution., Conclusion: The sensitivities of Technetium-99 and Gallium-67 to detect MOE are less favorable than was initially thought. Given this finding and their poor specificity, lack of anatomic resolution, unproven ability to detect disease resolution and variable availability, this review does not support the routine use of these studies in the management of MOE., Level of Evidence: N/A Laryngoscope, 130:1812-1816, 2020., (© 2019 The American Laryngological, Rhinological and Otological Society, Inc.)

Objectives/hypothesis: To describe the presentation and clinical course of cerebellopontine angle (CPA) and internal auditory canal (IAC) lipomas., Study Design: Retrospective cohort study at a tertiary academic referral center., Methods: All patients presenting with a CPA or IAC mass radiographically consistent with a lipoma on high-resolution magnetic resonance imaging (MRI) were identified. Data including presenting symptomatology, tumor characteristics, management strategy, and patient course were collected., Results: Between 1996 and 2012, 15 patients were diagnosed with a CPA or IAC lipoma at the authors' institution and were included in the analysis. The mean duration of radiological and clinical follow-up was 3.4 years and 5.1 years, respectively. Eight lesions were confined to the IAC, while seven involved the CPA. The median tumor size at diagnosis was 7.2 mm; one patient demonstrated tumor growth on serial MRI while the remaining subjects did not have radiological progression. The most common presenting symptoms were sensorineural hearing loss (40%) and tinnitus (33%); five patients were diagnosed after incidental discovery on MRI. Fourteen patients were managed with observation, while one subject underwent subtotal resection. None of the observed patients reported worsening symptoms at last follow-up., Conclusions: While rare, lipomas should be included in the differential diagnosis of CPA and IAC lesions. Owing to a generally benign clinical course and high morbidity associated with resection, microsurgery should only be considered in cases of definite tumor enlargement with intractable symptoms from mass effect. Careful radiological evaluation is critical for establishing an accurate diagnosis in order to prevent unnecessary morbidity associated with resection., (Copyright © 2012 The American Laryngological, Rhinological, and Otological Society, Inc.)

Objectives/hypothesis: To define the surgical treatment and outcomes of von Hippel-Lindau (VHL) disease-associated endolymphatic sac tumors (ELSTs), we analyzed consecutive VHL patients who underwent ELST resection., Study Design: Retrospective investigation of consecutive VHL patients who underwent resection of ELSTs at a clinical research center between 1999 and 2010., Methods: Analysis of serial clinical examinations, audiograms, imaging studies, and operative findings were analyzed., Results: Thirty-one consecutive patients with ELSTs (15 males, 16 females) underwent resection of 33 tumors (mean follow-up, 49.9 ± 48.0 months; range, 1.0-116 months). One patient had bilateral ELST resections and one patient underwent reoperation for recurrence. Mean age at surgery was 38.2 ± 10.2 years (range, 12-67 years). Whereas 29 ears (88%) had direct radiographic evidence of an ELST, four ears (12%) did not. Mean tumor size was 1.3 ± 1.1 cm (range, 0.2-5.2 cm). Whereas two patients (two ears, 6%) were asymptomatic, 29 patients (31 ears, 94% of ears) had associated audiovestibular symptoms, including sensorineural hearing loss (28 ears, 84%), tinnitus (24 ears,73%), and vertigo (21 patients, 68%). Postoperatively, hearing was stabilized (27) or improved (three) in 97% of 31 ears. Complete tumor resection was achieved in 30 ears (91% of 33 ears). Complications included cerebrospinal fluid leak in two ears (6%) and transient lower cranial nerve palsy in one ear (3%)., Conclusions: Surgical resection of ELSTs can be performed with hearing preservation and a reduction in audiovestibular dysfunction. Early surgical resection can prevent or decrease disabling audiovestibular symptoms, enhance the opportunity for complete resection, and preserve hearing., (Copyright © 2012 The American Laryngological, Rhinological, and Otological Society, Inc.)

Objective: To test whether early hearing loss (HL) is cochlear in origin in patients with vestibular schwannoma (VS)., Study Design: Retrospective case review in an academic tertiary referral center., Methods: A group of 19 VS patients with normal/symmetrical hearing and a group of 20 VS patients with mild HL (threshold at any tested frequency better than 45 dB HL) on the tumor ear side. Differences of the amplitudes of the distortion products of otoacoustic emissions (DPOAEs) between the tumor ear and the nontumor ear were studied at frequencies of 1, 1.4, 2, 2.8, and 4 kHz. The Wilcoxon test was used to compare the ears for both groups and to test for possible differences in tumor size between groups., Results: DPOAE amplitudes do not differ strongly between the ears in VS patients with normal/symmetrical hearing (two-sided P values: .050 at 1 kHz, .182 at 1.4 kHz, .378 at 2 kHz, .293 at 2.8 kHz, and .238 at 4 kHz) but are decreased compared with the nontumor ear at frequencies 1, 1.4, 2, and 2.8 kHz in VS patients with even mild HL (two-sided P values: .013 at 1 kHz, .007 at 1.4 kHz, .033 at 2 kHz, .010 at 2.8 kHz, and .156 at 4 kHz). Tumor size did not differ significantly between the two groups (P = .436)., Conclusion: Amplitudes of DPOAEs begin to decrease even at the early stages of HL in VS patients, which suggests a cochlear origin of early HL in these patients. DPOAEs may be used in a clinical setting to monitor progression of cochlear damage at the early stages of hearing impairment in VS patients.

Objective: Present four new cases of carcinoid tumor of the middle ear, two of which developed late recurrences and regional metastases. Review the literature to identify the clinical features, rate of recurrence, and incidence of metastasis of carcinoid tumor of the middle ear., Study Design: Retrospective chart review., Setting: Tertiary referral hospital., Patients: Eligibility criteria consist of a diagnosis of carcinoid tumor of middle ear., Intervention: Surgical excision of primary and metastatic disease., Main Outcome Measure: Clinical characteristics, rate of recurrence, and incidence of metastasis of carcinoid tumor of the middle ear., Results: Forty-six patients with carcinoid tumor of the middle ear are included in this report, 42 patients were identified from a review of the literature, and 4 new patients are presented. The most common presenting symptom was hearing loss. Surgical excision was the treatment with radical mastoidectomy being the most common procedure. Ten (22%) patients developed locally recurrent disease, and four (9%) developed regional metastases., Conclusions: Carcinoid tumor of the middle ear is an infrequent cause of a middle ear mass, with only 46 cases published. Despite previous assertions of benignancy, the findings of this study suggest that carcinoid tumor of the middle ear is indeed a potential low-grade malignancy with documented metastatic potential. Almost all middle ear adenomatous tumors ("adenoma" and "carcinoid") show evidence of neuroendocrine differentiation, and so at least some middle ear carcinoids ("adenomas") appear to represent well-differentiated neuroendocrine carcinomas. Presentation and symptoms are consistent with a middle ear mass and rarely include carcinoid syndrome. Surgical treatment is recommended and tailored to the extent of disease. Patients with carcinoid tumor of the middle ear require indefinite follow-up for possible recurrence or metastasis.

Objectives/hypothesis: Described is the first reported case of an angiofibrolipoma of the ear canal in a patient who presented with right-sided conductive hearing loss and a medial canal stenosis., Study Design: Case report., Results/conclusion: This variant of lipoma contains mature adipocytes, blood vessels, and dense collagenous tissue. The physical examination can be misleading, and the diagnosis requires histopathological examination. The patient was treated with complete surgical excision, tympanoplasty, canalplasty, and skin grafting to the external auditory canal. His pure-tone average improved from 37 to 11 dB, and his air-bone gap was closed completely. The lipoma has not recurred in the 6-month period following surgery.

Objective: To estimate the risk of loss of serviceable hearing during the conservative management of vestibular schwannomas., Study Design: Retrospective case review., Methods: Twenty-five patients with a radiological diagnosis of unilateral vestibular schwannoma were managed conservatively for a mean duration of 43.8 months (range, 12-194 mo). The pure-tone average (PTA) (0.5, 1, 2, and 3 kHz) and speech discrimination scores (SDS) were measured at regular intervals throughout the entire duration of follow-up. Serviceable hearing was defined using two criteria: 70% SDS/30 dB PTA (the 70/30 rule) and 50% SDS/50 dB PTA (the 50/50 rule). The size and growth rate of tumors were determined according to the American Academy of Otolaryngology-Head and Neck Surgery guidelines (1995). Intervention was recommended if there was evidence of continuous or rapid radiological tumor growth, and/or increasing symptoms or signs suggestive of tumor growth., Results: The risk of loss of serviceable hearing for the total group was 43% using the 70/30 rule and 42% using the 50/50 rule. Tumor growth was considered significant (> 1 mm) in 8 tumors (32%) and nonsignificant in 17 (68%). The risk of loss of serviceable hearing for the tumor-growth group was 67% using the 70/30 rule and 80% using the 50/50 rule. In contrast, the risk of loss of serviceable hearing for the no tumor-growth group was 25% using the 70/30 rule and 14% using the 50/50 rule. No audiological factors predictive of tumor growth were identified., Conclusions: There is a significant risk of loss of serviceable hearing during the conservative management of vestibular schwannomas. This risk appears to be greater in tumors that demonstrate significant growth.

Previous reports of endocrine activity of glomus jugulare tumors have been of pheochromocytomas but not carcinoid apudomas. This paper is to alert the otolaryngologist to the presence of carcinoid apudomas in glomus jugulare tumors. A patient with a long history of explosive diarrhea, facial flushing, and violent headaches, the symptom of carcinoid syndrome, was cured after removal of his large glomus jugulare tumor. The diagnosis of carcinoid syndrome is made with a good history and confirmed with a 24 hour urine collection for 5 Hydroxy indole acetic acid (5 Hiaa). a series of patients treated at the Johns Hopkins Hospital for glomus jugulare tumors is reviewed for the presence of endocrine activity and the literature on endocrine active glomus jugulare tumors is presented. Preoperative evaluation for the presence of both pheochromocytoma and carcinoid apudoma is recommended for all patients with glomus jugulare tumors.

Brain stem auditory evoked potential (BAEP) testing has been demonstrated to be a useful diagnostic tool in the presence of severe or complete sensorineural hearing loss. From a series of 38 patients with tumors in the vicinity of the VIIIth nerve, 13 patients with tumors larger than 2 cm had abnormal BAEP results contralateral to the involved side. The abnormalities observed on the noninvolved side included prolonged peak latencies, reduced amplitudes, poor wave shape and poor response stability. The significance of these findings is twofold: 1. Large tumors may disrupt BAEP test results on the side contralateral to the lesions, 2. Important information may be obtained on patients having complete sensorineural hearing loss on the involved side.

Malignant external otitis (necrotizing otitis externa) is an infectious process which occurs most often in middle-aged and elderly diabetic patients and is characterized by cultures positive for Pseudomonas and granulation tissue at the junction of the bony and cartilaginous portion of the external auditory canal. Epidermoid carcinoma of the external auditory canal is also seen most often in the middle-aged and elderly population. To the best of our knowledge, no case of acute necrotizing otitis externa occurring concomitantly with epidermoid carcinoma of the external auditory canal has been documented. A case is presented and discussed here and the importance of biopsy of the external auditory canal is stressed. Theoretical considerations of the possible relationship between these two disorders are discussed. This case illustrates the need to discontinue the use of the term "malignant external otitis" and replace it with the term "necrotizing otitis externa."

Two cases of nonkeratinizing squamous cell carcinoma (transitional cell carcinoma), both of which arose in the Eustachian tube, and two cases of neurilemoma of the nasopharynx are reported. All four patients presented with unilateral serous otitis media. The duration of the unilateral serous otitis media in these patients ranged from 1 1/2 to 8 years. This emphasizes the fact that adult patients with unilateral serous otitis media without a history of an upper respiratory tract infection or of barotrauma should undergo thorough examination to rule out the presence of an unusual underlying pathologic condition.

The occurrence of vascular perturbations in the tympanogram in association with glomus tumors has previously been noted in the literature. We have broadened the scope of this method of diagnosis in the study of 3 glomus tympanicum and 3 glomus jugulare tumors, 1 dehiscent high jugular bulb, 1 aberrant carotid artery in the middle ear and 2 cases of pulsatile tinnitus of vascular origin. Analysis of illustrative tympanograms at standard and at increased sensitivity (x 20) obtained in these cases are presented. The following factors as they relate to the mechanism of recording the vascular perturbations are presented: air pressure and/or presence of fluid in the middle ear; compliance of the tympanic membrane and ossicular chain as affected by the mass, size and degree of vascularity of the mass and presence of extraneous sounds. A new method of recording the vascular perturbations utilizing a time-base generator affixed to an X-Y plotter is described. It is the purpose of this study to demonstrate the utility of obtaining tympanograms at standard and at increased sensitivity in the evaluation of vascular middle ear lesions and tinnitus of vascular origin.

An ultrastructural study of the interface between aural cholesteatoma and adjacent bone was performed on specimens obtained from human and experimental gerbilline cholesteatoma. When an enlarging cholesteatoma contacted bone, a large number of monocytes and macrophages accumulated in the area of contact. The intervening middle ear mucosa degenerated and bone erosion occurred. Anatomic evidence is provided which indicates that bone erosion in both human and experimental cholesteatoma occurred as a result of the action of multinucleated osteoclasts. These osteoclasts had the ultrastructural appearance of normal osteoclasts with a well developed ruffled border and large numbers of mitochondria. These cells stained readily with acid phosphatase stains. Although many other mononuclear cells were seen in the vicinity of the eroded bone, only the multi-nucleated osteoclasts were associated with disappearance of the bone surface. Osteoclasts were commonly found in experimental cholesteatoma, but were infrequent in human cholesteatoma biopsies, presumable because patients undergoing cholesteatoma surgery ara often treated with topical installations of corticosteroids which inhibit osteoclasts.

We reviewed the findings in 1,220 cases of sudden sensori-neural hearing impairment encountered over a nine-year period at the Otologic Medical Group. A thorough neuro-otologic evaluation is indicated in each case. Ten patients had an acoustic tumor. A vasodilator regimen was prescribed in 380 cases and resulted in hearing improvement in 40%. Favorable prognostic findings were a low tone loss and absence of vertigo.