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A 52-year-old man presented to our emergency department with an acute onset of right-sided facial nerve (FN) palsy of House-Brackmann grade V. Electroneurography (ENoG) was conducted with no response at the right FN, as compared with the left FN (0%). We performed a biopsy of the right middle ear mass and histological studies showed the tumor to be neuroendocrine tumors (NET) of the middle ear. We resected the tumor with canal wall down mastoidectomy and reconstructed the posterior meatal wall with soft tissue. Three months after surgery, the FN paralysis had improved with House-Brackmann grade II. We reviewed cases of NET with FN palsy, and nine patients, including our case, have been reported. Our case is the first report of ENoG for the description of FN palsy due to NET. Although the ENoG value was 0%, it was remarkably improved by surgery. The other cases of NET patients with FN palsy also recovered FN function after surgery. These results suggest that it is recommended to perform the total resection of the tumor to improve the FN function., (Copyright © 2020. Published by Elsevier B.V.)

The prognosis of advanced temporal bone cancer is poor, because complete surgical resection is difficult to achieve. Chemoradiotherapy is one of the available curative treatment options; however, its systemic effects on the patient restrict the use of this treatment. A 69-year-old female (who needed peritoneal dialysis) presented at our clinic with T4 left external auditory canal cancer and was treated with cetuximab plus radiotherapy (RT). The primary lesion showed complete response. The patient is currently alive with no evidence of disease two years after completion of the treatment and does not show any late toxicity. This is the first advanced temporal bone cancer patient treated with RT plus cetuximab. Cetuximab plus RT might be a treatment alternative for patients with advanced temporal bone cancer., (Copyright © 2017 Elsevier B.V. All rights reserved.)

Introduction: Dermoids of the Eustachian tube are rare benign developmental tumours that typically occur in female children. General consensus of classification and nomenclature has still not been reached. The treatment of choice consists of a radical surgical excision. Several approaches have been described and few cases are reported in literature. A gross total resection is now safely achievable through an endoscopic mini-invasive approach., Materials and Methods: We have reported a case of dermoid pedicled in the left Eustachian tube and resected with a pure endoscopic transnasal approach. A review of the literature was performed., Case Study: The aim of this paper is to present the first case of pure endoscopic transnasal resection in a 4-day-old infant as emergency treatment of Eustachian tube dermoid presenting an acute respiratory failure at birth., Conclusion: The endoscopic transnasal resection is a safe and feasible technique in selected dermoids of the Eustachian tube, when the middle ear is not involved. This approach could be used also in new-born children, decreasing the morbidity of the classic surgical treatment and avoiding the risk of craniofacial alterations., (Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.)

Objective: The case of a thirteen-year-old woman showing an atypical teratoid/rhabdoid tumor (AT/RT) primarily occurred in the internal auditory canal was presented., Results: There was a delay in diagnosing AT/RT because of the first histological diagnosis of benign neurofibroma. If we had changed the surgical approach to one which was middle cranial fossa-based or translabyrinthine in the second or third operation, we might have reached an earlier final diagnosis. Although we faced a dilemma about whether to sacrifice facial nerve function for dissection of the tumor, we should have considered the possibility of malignancy at an earlier stage., Conclusion: This is a case report of AT/RT in the internal auditory canal presenting with progressive hearing loss as the initial symptom. Although no previous reports of AT/RT primarily occurring in the internal auditory canal are existent, this rare form of the disease should be considered in future evaluations as a differential diagnosis for internal auditory canal tumor., (Copyright © 2014 Elsevier Ireland Ltd. All rights reserved.)

Intravestibular lipoma (IVL) and intravestibular schwannoma (IVS) are rare tumors occupying the intravestibular space. Patients with IVL or IVS complain of hearing impairment, tinnitus or recurrent rotatory vertigo. Therefore, the clinical practitioner could misdiagnose them as sudden sensorineural hearing loss or Meniere's disease. Since delayed diagnosis and treatment could lead to more severe and refractory symptoms, clinicians should have a high index of suspicion for early diagnosis. Recent advancements in imaging diagnostic tools such as computed tomography and magnetic resonance imaging have facilitated the correct diagnosis of these intravestibular tumors without surgical removal. Presently, we report two different kinds of intravestibular tumors of lipoma and schwannoma which manifest different clinical course and treatment strategies., (Copyright © 2011 Elsevier Ireland Ltd. All rights reserved.)

Objective: We report an extremely rare case of thymoma which developed middle ear metastasis along with acute sensorineural hearing loss in the contra ear., Method: We present a case report and a review of the world literature concerning thymoma metastases to the middle ear., Results: A 54-year-old female patient with thymoma who developed middle ear metastasis along with acute sensorineural hearing loss in the contra ear., Conclusion: We have not found out thymoma metastases to the middle ear in the past., (Copyright © 2011 Elsevier Ireland Ltd. All rights reserved.)

Objective: Glomus tumors are slow-growing benign lesions and represent the most common primary neoplasms of the middle ear. The objective of the present study is to report our surgical strategy in the management of glomus tympanicum tumors., Methods: Between December 1988 and July 2008, 68 patients with histologically confirmed glomus tympanicum tumor underwent surgical treatment. The follow-up of the series ranged from 6 to 194 months (mean, 33.4 months)., Results: Distribution of tumors according to Fisch and Mattox classification was as follows: type A, 44 cases (64.7%); type B, 24 cases (35.3%). All of the 44 Class A tumors were safely removed via either a stapedectomy-type transcanal approach or a retroauricolar-transcanal approach. Five patients with Class B tumors were operated on through a transmastoid approach. Nineteen patients with larger Class B tumors underwent a subtotal petrosectomy with blind sac closure of the external auditory canal and middle ear obliteration. Gross total tumor removal was achieved in all 68 cases. In one case (1.4%) there was recurrence after 9 years, for which the patient was re-operated. No residual/recurrence has been detected on computed tomography in the rest of the patients., Conclusion: Surgical excision of glomus tympanicum tumors is a safe procedure, allowing total tumor removal with minimal morbidity, a low recurrence rate, and a low complication rate., (Copyright (c) 2010 Elsevier Ireland Ltd. All rights reserved.)

The management of the facial nerve in tumorous temporal lesions is particularly challenging due to its complex anatomic location and potential postoperative complications, including permanent facial paralysis. The most important concern regarding surgical treatment of a tumorous temporal lesion is the inevitable facial paralysis caused by nerve injury during the tumor removal, especially in patients with minimal to no preoperative facial nerve dysfunction. We describe successful four cases in which various surgical techniques were developed for the preservation of the facial nerve in treatment of intratemporal tumorous lesions., (Copyright (c) 2009 Elsevier Ireland Ltd. All rights reserved.)

We present a case of meningeal carcinomatosis with bilateral hearing loss secondary to a rectal adenocarcinoma. A 60-year-old woman developed progressive loss of hearing in the left ear 19 months after an abdominoperineal resection for an adenocarcinoma of the rectum. Three months after the onset of left hearing loss, she visited our hospital. Pure tone audiometry revealed profound sensorineural hearing loss in the left ear and mild sensorineural hearing loss in the right ear. Gadolinium-enhanced MRI revealed tumor in the left internal auditory canal and cerebellopontine angle and enhancement in the right internal auditory canal. Six days after the first examination, pure tone audiometry revealed profound loss of hearing in the right ear. DPOAE of the right ear were still detected 6 days after the first examination, but were clearly decreased 9 days after it, and reached noise level 10 days after it. Gadolinium-enhanced MRI revealed rapid growth of the tumor of the right internal auditory canal and cerebellopontine angle. We clearly demonstrate here the rapid course of hearing loss using pure tone audiometry, MRI, and DPOAE.

We report a case of endolymphatic sac tumor (ELST). A 48-year-old female had recurrent and slowly progressive hearing loss, accompanied with dizziness like Meniere's disease. A tumor was located around the semicircular canals, and was detected on CT and MRI. The patient underwent total removal of the tumor using a transmastoid approach. Histopathological examinations agreed with features of an ELST. The tumor was highly suspected to have originated from the rugose portion of the endolymphatic sac or the endolymphatic duct, based on surgical and imaging studies. Structure of the membranous labyrinth was preserved regardless of the existence of the tumor around semicircular canals with bone destruction. ELSTs seem to have an osteolytic or osteophilic nature, by examining patterns of tumor infiltration.

Middle-ear ceruminous adenomas are rare benign neoplasms arising from the epithelium of the middle ear. Progressive hearing loss, ear fullness and tinnitus are common symptoms of this tumour; facial nerve paresis and vestibular disturbances occur very infrequently. We present two cases of middle-ear ceruminous adenomas, one showed rapid unilateral hearing loss with aural fullness, followed by purulent aural discharge and vertigo. In the second case, the disease affected an already deaf ear and the only symptom of the disease was increasing vertigo. The clinical features, intraoperative findings, and histological and radiological findings are presented. The cases are compared to those described in the literature.

Purpose: We chronologically investigated whether the hearing preservation (HP) rate improved or not in vestibular schwannoma (VS) surgery., Subjects and Methods: HP surgery has been attempted in 127 VS patients with pre-operative class A and B hearing from 1976 to 1999. The HP rate was chronologically compared with hearing level and tumor size., Results: The preservation rate of pre-operative class A hearing which was preserved post-operatively as class A has recently been increasing especially in patients with an intracanalicular (IC) tumor. This rate was improved to 67% from 1995, although this was 33% from 1989 to 1994. However, the preservation rate of pre-operative class B in patients with an IC tumor and that of pre-operative class A hearing which was preserved as class A in patients with a larger tumor extending into the posterior fossa, have not improved over the last 10 years., Conclusion: These results indicate that the improvement of the HP rate is mainly due to the increased number of patients with an IC tumor with class A pre-operative hearing. In patients with a tumor of 4-20 mm in size, especially in those showing pre-operative class B hearing, there may be histologically some limitations such as gliosis in the cochlear nerve in the preservation rate of good quality (class A or B) of hearing.

A case of cavernous hemangioma in the internal auditory canal was reported. The chronology of this case was similar as one of Ménière's disease. The neurotological findings in this case were no specific findings to differentiate cavernous hemangioma from acoustic neurinoma. The high-resolution computed tomography (HRCT) revealed calcium stippling in the lesions, whereas magnetic resonance imaging (MRI) demonstrated a low signal intensity on both T1- and T2-weighted images. Comparison of HRCT and MRI findings is most useful for differential diagnosis of intracanalicular tumors.

Intracochlear schwannoma was found in the temporal bone of a 85-year-old man in whom audiometric study, 26 days before death, had shown total deafness in the left ear. The tumor occupied the entire lumen of the cochlea in the basal turn involved Rosenthal's canal, but it occupied only the scala tympani in the second turn. Intralabyrinthine schwannomas are difficult to diagnose by clinical examination. They were discovered accidentally during destructive labyrinthectomy for presumed Ménière's disease or discovered incidentally by postmortem temporal bone pathology. Although intralabyrinthine schwannomas are a rare occurrence and cannot usually be diagnosed without surgery or postmortem histopathology, it is important to suspect the possibility of their existence in differential diagnosis of atypical Ménière's disease or unilateral idiopathic progressive deafness. Long-term follow-up is obviously necessary to exclude the tumor.