Hearing Loss

Paraganglioma presenting as cholesterol granuloma of the petrous apex

September 18, 2013     Selena E. Heman-Ackah, MD, MBA; Tina C. Huang, MD
article

Abstract

We report the unique finding of a petrous apex cholesterol granuloma associated with a paraganglioma, also known as a glomus jugulare tumor, in a 52-year-old woman who presented to our department with pulsatile tinnitus, hearing loss, aural fullness, and disequilibrium. She had been treated for a petrous apex cholesterol granuloma 20 years earlier, at which time she had undergone drainage of the granuloma via subtotal petrous apicectomy. When she came to our facility approximately 20 years later, she had signs and symptoms consistent with a jugular paraganglioma, which was likely to have been present at the time of her initial presentation for the cholesterol granuloma. In fact, microscopic bleeding from the paraganglioma might have led to the formation of the cholesterol granuloma. The metachronous presentation of these two entities, which to our knowledge has not been reported previously in the literature, indicates the potential association of paragangliomas with the formation of cholesterol granulomas of the petrous apex.

Medial canal fibrosis

September 18, 2013     Joseph A. Ursick, MD; John W. House, MD
article

Medial canal fibrosis is an uncommon condition characterized by progressive stenosis of the bony external auditory canal.

Transient auditory dysfunction: A description and study of prevalence

August 21, 2013     Laurence Maximilian Almond, MB ChB; Ketul Patel, MB ChB; and Darius Rejali, MB ChB
article

Abstract

Transient auditory dysfunction (TAD) is a previously undescribed symptom complex of unknown cause. It is characterized by short-lasting sensorineural hearing loss (unilateral or bilateral), it is associated with tinnitus, it resolves completely within minutes, and it is not accompanied by vestibular symptoms. We conducted a cross-sectional prospective study to define TAD, find its prevalence, and discuss its significance. Two hundred healthy subjects between the ages of 16 and 49 years were surveyed using a questionnaire. Of these subjects, 41 (20.5%) reported experiencing symptoms of TAD. The mean number of episodes was 5.9 times per month, the mean duration was 41 seconds, and 80% experienced concomitant tinnitus. We conclude that TAD is a common finding in a healthy population. This may have implications for the pathogenesis of sudden-onset sensorineural hearing loss. Further longitudinal studies and detailed audiologic evaluation of patients with TAD are required to ascertain the significance, etiology, and pathophysiology of this condition.

Cicatricial external auditory canal stenosis caused by ectodermal dysplasia: Rapp-Hodgkin syndrome

June 11, 2013     Amanda B. Sosulski, MD and James D. Hayes, MD
article

Abstract

We present a case of recurrent cicatricial stenosis of the external ear canals caused by ectodermal dysplasia, specifically Rapp-Hodgkin syndrome, in a 45-year-old woman. No form of medical or surgical management has produced durable patency of the patient's ear canals, and her hearing loss is being managed with hearing aids. Topical management of the recurring external otitis slows the process but has been unsuccessful in preventing restenosis of both external auditory canals.

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.

An extruded ossicular prosthesis

February 25, 2013     Willis S.S. Tsang, FRCSEd(ORL); Michael C.F. Tong, MD(CUHK); C. Andrew van Hasselt, M Med(Otol)
article

Extrusion of ossicular prostheses results from postoperative tympanic membrane medialization. The incorporation of cartilage grafting material is recommended to minimize extrusion.

Complicated coexisting pyogenic and tuberculous otitis media affecting the temporozygomatic, infratemporal, and parotid areas: Report of a rare entity

January 24, 2013     Tripti Brar, MBBS, MS, DNB; Sumit Mrig, MBBS, MS, DNB; J.C. Passey, MS; A.K. Agarwal, MS; Shayma Jain, MD
article

Abstract

We report an unusual case in which a 28-year-old woman presented with a long-standing history of ear discharge, hearing loss, facial weakness with ipsilateral facial swelling and cellulitis, a postauricular fistula, and an abscess of the temporozygomatic, infratemporal, and parotid areas. The pus stained positive for bacteria and acid-fast bacilli, and culture was positive for Proteus vulgaris and mycobacteria. Based on these findings, a diagnosis of tuberculous otitis media with complications was made. Computed tomography showed extensive destruction of the tympanic and mastoid part of the temporal bone, as well as lytic lesions in the skull. The patient was placed on antituberculosis drug therapy. Although her facial nerve palsy and hearing loss persisted, she otherwise responded well and did not require surgery.

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.

Hearing loss secondary to a nasopharyngeal retention cyst

October 31, 2012     Enrique Palacios, MD, FACR; Michael Ellis, MD; Harold Neitzschman, MD, FACR
article

Resection is generally not indicated for small, asymptomatic pharyngeal cysts. Symptomatic cysts, on the other hand, can be treated with aspiration or a complete transoral resection, particularly if the lesion is large.

Cholesterol granuloma

October 8, 2012     Joseph A. Ursick, MD; Derald E. Brackmann, MD
article

 Cholesterol granulomas can cause bony expansion and typically become symptomatic when they encroach upon adjacent cranial nerves.

Bilateral posterior semicircular canal dehiscence in the setting of Hallermann-Streiff syndrome

September 7, 2012     John C. Goddard, MD; Eric R. Oliver, MD; Ted A. Meyer, MD, PhD
article

Abstract

Hallermann-Streiff syndrome, also known as oculomandibulofacial syndrome, is a rare congenital disorder affecting growth and cranial, dental, ocular, pilocutaneous, and mental development. In addition to routine audiologic testing in patients with this syndrome, high-resolution computed tomography of the temporal bones should be performed in those with documented or suspected sensorineural hearing loss. Cochlear implantation may be considered, as in other children with syndromic hearing loss and certain inner ear abnormalities, if the appropriate audiologic, psychosocial, and medical criteria are met. The current case report radiographically and clinically characterizes inner ear dysplasia in an 8-year-old patient with Hallermann-Streiff syndrome. High-resolution computed tomography of the temporal bones revealed a hypoplastic bony island between the vestibule and horizontal semicircular canals, as well as incomplete bony coverage of the posterior semicircular canal crura bilaterally. To our knowledge, this is the first report of a pediatric patient demonstrating bilateral posterior semicircular canal dehiscence.

Bilateral hemotympanum secondary to septoplasty

September 7, 2012     Jae Hoon Lee, MD
article

Hemotympanum can be caused by temporal bone fractures, barotrauma, administration of anticoagulants, hematologic disorders, and chronic otitis media secondary to the formation of granulation tissue or a cholesterol granuloma. It can also occur secondary to nasal packing or spontaneous epistaxis, as well as eustachian tube dysfunction.

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