Inner ear

Temporal bone fracture

January 21, 2014     Danielle M. Blake, BA; Senja Tomovic, MD; Robert W. Jyung, MD
article

Transverse fractures account for approximately 20% of temporal bone fractures. They occur secondary to frontal or occipital head trauma, and they run perpendicular to the petrous pyramid.

Endolymphatic sac tumor

April 17, 2013     Lester D.R. Thompson, MD
article

Behcet disease as a cause of hearing loss: A prospective, placebo-controlled study of 29 patients

March 24, 2013     Ozgur Kemal, MD; Yucel Anadolu, MD; Ayse Boyvat, MD; and Ahmet Tataragasi, AuD
article

Abstract

We conducted a prospective, placebo-controlled study to determine the incidence and severity of inner ear involvement and hearing loss in patients with Behçet disease. Our study population was made up of 29 patients with Behçet disease and 28 healthy controls. Audiometric pure-tone thresholds and transient evoked otoacoustic emission (TEOAE) levels were determined in both groups. The main outcome measures were pure-tone audiometry (PTA) levels and TEOAE levels in the two groups. PTA detected a sensorineural hearing loss in 10 of the 29 patients (34.5%). The difference in audiometric findings between the two groups was statistically significant at 1, 2, 4, and 8 kHz (p ≤ 0.0498). A comparison of TEOAE levels revealed that the difference in sound-to-noise ratio between the two groups was not significant at 1, 1.5, 2, and 3 kHz, but it was significant in 4 kHz (p = 0.02), and the difference in reproducibility between the two groups was significant at 2 and 4 kHz (p ≤ 0.03). We conclude that all patients with Behçet disease should be screened for hearing impairment and subsequently treated if an impairment is discovered.

Primary mucosal melanoma arising from the eustachian tube with CTLA-4, IL-17A, IL-17C, and IL-17E upregulation

January 24, 2013     Calvin Wei, MD; Sasis Sirikanjanapong, MD; Seth Lieberman, MD; Mark Delacure, MD; Frank Martiniuk, PhD; William Levis, MD; Beverly Y. Wang, MD
article

Abstract

Primary malignant melanoma arising from the eustachian tube is extremely rare. We report the case of a 63-year-old white man who presented with a 1-month history of left-sided hearing loss and aural fullness. Flexible fiberoptic laryngoscopy detected a blue-purple mass that appeared to arise from the left lateral nasopharynx. Computed tomography demonstrated an enhancing mass arising from an orifice of the left eustachian tube. The tumor was debulked endoscopically and was confirmed to have originated in the left eustachian tube. Histologically, the tumor was made up of heavily pigmented pleomorphic spindle cells with frequent mitoses. The tumor cells were immunohistochemically positive for S-100 protein, HMB-45, Melan-A, and PNL-2. The final diagnosis was a mucosal malignant melanoma. We also performed a nested polymerase chain reaction assay for several genes of interest, including CTLA-4, IL-17A, IL-17B, IL-17C, IL-17D, IL-17E, IL-17F, PLZF, Foxp3, RORγt, CD27, and CD70. These genes have been studied mainly in cutaneous melanomas, especially for the development of immunotherapy, but only very limited studies have been done on mucosal melanomas. Our investigation found upregulation of CTLA-4, IL-17A, IL-17C, and IL-17E. Based on our finding of CTLA-4 upregulation, it may be suggested that our patient might have had low antitumor immunity and that he might have benefited from CTLA-4 blockade. On the other hand, upregulation of IL-17A and IL-17E might reflect increased antitumor immunity, which could suggest that patients with a mucosal melanoma might benefit from immunomodulators associated with the effect of Th17. These genes also have great potential to help melanoma patients obtain tailored treatment, and they can be used as biomarkers for predicting prognosis.

Recurrent meningitis in an adult secondary to an inner ear malformation: Imaging demonstration

March 31, 2012     Nisar Ahmad Wani, MD, Aijaz Rawa, MD, Umar Qureshi, MD, Tasleem Kosar, DMRD, and Irfan Robbani, MD
article

Abstract

Congenital labyrinthine dysplasia with a translabyrinthine cerebrospinal fluid (CSF) fistula may be an anatomic cause for recurrent meningitis. This condition is usually seen in children aged 5 to 10 years who present with sensorineural hearing loss (SNHL) and CSF discharge through the nose or ear, with or without recurrent meningitis. Multidetector-row computed tomography (MDCT) and high-resolution T2-weighted magnetic resonance imaging (MRI) of the petrous portion of the temporal bone can help to diagnose this abnormality. We report a case of translabyrinthine CSF fistula in an adult-a 30-year-old man-who presented with recurrent pneumococcal meningitis, a long history of a clear nasal discharge, and evidence of SNHL. MDCT and MRI of the temporal bone demonstrated a cystic-appearing cochleovestibular malformation (an incomplete partition type I) in the right inner ear. Imaging also showed an absence of the basal turn of the cochlea and the cribriform membrane at the lateral end of the right internal auditory canal, which was shorter and narrower than normal. Evidence of fluid in the right middle ear suggested a CSF fistula.

Dexamethasone inner ear perfusion for subclinical endolymphatic hydrops

February 1, 2009     Herbert Silverstein, MD, FACS, Mathew Farrugia, DO, and Mark Van Ess, DO
article

Abstract

We suggest that the term subclinical cochlear hydrops be used to describe patients presenting with aural fullness, stuffiness, or ear pressure when all other diagnoses have been excluded. We reviewed the charts of 20 patients who had presented with a chief complaint of aural fullness, stuffiness, or pressure and who underwent perfusion of the inner ear for 1 month with dexamethasone 4 or 10mg/ml. The symptoms improved in 13 (68.4%) of 19 patients after treatment. None of these patients reported a worsening of symptoms, and no patient had worse hearing after perfusion. Complications were infrequent, and patients tolerated the treatment well. Perfusion of the inner ear appears to be a safe procedure with little risk.

Is Susac syndrome (microangiopathy of the inner ear, retina, and central nervous system) an underdiagnosed cause of sensorineural hearing loss?

June 30, 2008     Richard Westreich, MD and Sujana Chandrasekhar, MD
article

Abstract

Susac syndrome is a rare disorder caused by the development of microangiopathic lesions in the inner ear, retina, and central nervous system. Although relatively few cases have been reported in the literature, this diagnosis is becoming more frequent as physician awareness increases. A high index of suspicion is needed to make the diagnosis because the signs and symptoms of Susac syndrome are those of a wide range of differential diagnoses. Otolaryngologists should be familiar with this syndrome because hearing loss may be the initial presenting symptom. We describe a new case of Susac syndrome to further promote physician awareness, and we discuss the options for diagnosis and treatment.

Endaural labyrinthectomy

March 31, 2008     Michael M. Paparella, MD
article

Sudden onset of sensorineural hearing loss secondary to intralabyrinthine hemorrhage: MRI findings

March 1, 2008     William M. Rosado Jr., MD and Enrique Palacios, MD, FACR
article

Noise-induced perilymph fistula

April 1, 2006     Brian Kung, MD; Robert T. Sataloff, MD, DMA
article
Abstract
Perilymph fistulae are difficult to diagnose because they present with a wide variety of signs and symptoms, they are associated with many etiologies, and they often mimic other conditions. In this article, we describe a case of perilymph fistula that featured one of its more rare causes: acoustic trauma-specifically, damage from a loud blast from the siren of a fire engine. We also review the literature and discuss the difficulties of diagnosis and treatment and the possible mechanisms by which acoustic trauma and other etiologies cause perilymph fistulae.

Preoperative computed tomography may fail to detect patients at risk for perilymph gusher

December 1, 2005     Michael D. McFadden, MD; Jason G. Wilmoth, MD; Anthony A. Mancuso, MD; Patrick J. Antonelli, MD
article
Abstract
Perilymph gusher (PG) is a very rare occurrence that can lead to an adverse outcome during inner ear surgery. In the absence of a family history of X-linked mixed deafness syndrome, surgeons may have difficulty determining if a patient is at risk preoperatively. Radiographic imaging is often performed in an attempt to identify such a possibility, but there are few data to support the value of negative studies. We conducted a retrospective study of 3 cases of PG in which findings on preoperative high-resolution computed tomography (CT) of the temporal bone had been interpreted as normal. We reviewed these CTs to discern if they did in fact demonstrate any abnormalities that might have indicated a risk of PG, and we found that the original radiologist had missed a dilated internal auditory canal and a deformity of the cochlear modiolus in the affected ear of 1 of these patients. No abnormality was detected on review of the CTs of the other 2 patients. Therefore, we conclude that negative CT findings do not necessarily rule out a risk of PG.
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