Otology

Cavernous hemangioma of the external auditory canal

April 17, 2013     Min-Tsan Shu, MD; Kang-Chao Wu, MD; Yu-Chun Chen, MD
article

The differential diagnosisfor cavernous hemangioma of the external auditory canal includes attic cholesteatoma with aural polyp, glomus tumor, arteriovenous malformation, granulation tissue, and carcinoma of the EAC.

Chondromyxoid fibroma of the mastoid portion of the temporal bone: MRI and PET/CT findings and their correlation with histology

April 17, 2013     Noeun Oh, MD; Azita S. Khorsandi, MD; Sophie Scherl, BA; Beverly Wang, MD; Bruce M. Wenig, MD; Spiros Manolidis, MD; Adam Jacobson, MD
article

Abstract

We report a very rare case of a chondromyxoid fibroma of the mastoid portion of the temporal bone in a 38-year-old woman who presented with left-sided hearing loss. Magnetic resonance imaging identified an expansile mass in the left mastoid bone with a heterogeneous hyperintense signal on T2-weighted imaging and peripheral enhancement. Subsequent positron emission tomography/computed tomography identified erosive bony changes associated with hypermetabolism. The patient underwent an infratemporal fossa resection with a suboccipital craniectomy/cranioplasty. We briefly review the aspects of this case, including a discussion of the differential diagnosis and the correlation between histologic and imaging findings.

Mastoid osteoma: A case report and review of the literature

March 24, 2013     Jeffrey Cheng, MD; Roberto Garcia, MD; Eric Smouha, MD
article

Abstract

We describe the case of a 22-year-old woman who presented with a slowly growing osseous lesion of the mastoid cortex. On computed tomography, the lesion was found to involve the mastoid cortex, with which it demonstrated similar attenuation. The indications for treatment in this case were the patient's sensation of a mass effect, the encroachment of the mass onto the external auditory meatus, and a cosmetic deformity. The tumor was removed in its entirety via a postauricular approach. Findings on histopathologic examination were consistent with a compact osteoma. Mastoid osteomas are rare, benign tumors. If their growth significantly occludes the meatus, they may cause cosmetic deformities, conductive hearing loss, and recurrent external ear infections. Several other osseous lesions of the temporal bone should be considered in the differential diagnosis. The etiology of mastoid osteomas is poorly understood. Surgical management can be undertaken with minimal postoperative morbidity.

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.

Cerebrospinal fluid leak of the fallopian canal

March 24, 2013     Karen B. Teufert, MD; William H. Slattery, MD
article

Abstract

Spontaneous cerebrospinal fluid (CSF) leaks from the fallopian canal are extremely rare, as only a few cases have been reported in the world literature. We describe a case of spontaneous CSF otorrhea through an enlarged geniculate fallopian canal. The patient was a 45-year-old woman who presented with a history of CSF rhinorrhea and otorrhea from the right ear. Myringotomy and tube insertion revealed CSF otorrhea. Contrast-enhanced computed tomography revealed that the geniculate fossa was smoothly enlarged (demonstrating remodeling of bone). A middle fossa craniotomy with temporal bone exploration was performed. Intraoperative inspection detected the presence of a fistula secondary to a lateral extension of the subarachnoid space through the labyrinthine segments of the fallopian canal. We discuss the management of this unusual finding, which involves sealing the fistula while preserving facial nerve function.

Retention cyst in chronic otitis media

March 24, 2013     Min-Tsan Shu, MD; Kang-Chao Wu, MD; Yu-Chun Chen, MD
article

The retention cyst originates from the obstruction of a glandular structure and contains fluid, while the cholesteatoma contains keratinizing squamous epithelium.

Middle ear metastasis from dormant breast cancer as the initial sign of disseminated disease 20 years after quadrantectomy

March 24, 2013     Teresa Pusiol, MD; Ilaria Franceschetti, MD; Francesca Bonfioli, MD; Francesco Barberini, MD; Giovanni Battista Scalera, MD; Irene Piscioli, MD
article

Abstract

We describe an unusual case of breast cancer metastatic to the middle ear in a 71-year-old woman. The metastasis was the initial sign of disseminated disease 20 years after the patient had undergone a quadrantectomy for her primary disease. Computed tomography (CT) demonstrated the presence of an intratympanic mass with a soft-tissue density that was suggestive of chronic inflammation. The patient underwent a canal-wall-down tympanoplasty. When a brownish mass was found around the ossicles, a mastoidectomy with posterior tympanotomy was carried out. However, exposure of the tumor was insufficient, and therefore the posterior wall of the ear canal had to be removed en bloc. Some tumor was left on the round window membrane so that we would not leave the patient with a total hearing loss. Our case highlights the limitations of CT and magnetic resonance imaging in differentiating inflammatory and neoplastic lesions.

Recurrent post-tympanostomy tube otorrhea secondary to aerobic endospore-forming bacilli: A case report and brief literature review

February 25, 2013     James J. Jaber, MD, PhD; Matthew L. Kircher, MD; Eric Thorpe, MD; Ryan G. Porter Sr., MD; John P. Leonetti, MD; Sam J. Marzo, MD
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Abstract

We report a unique case in which a 57-year-old man with an 8-year history of late recurrent post-tympanostomy tube otorrhea (PTTO) was found to harbor Bacillus subtilis, an aerobic endospore-forming bacillus that is typically resistant to chemical and physical agents because of its unique life cycle. Removal of the patient's tympanostomy tube resulted in complete resolution of his long-standing otorrhea. We also review the etiologies of and treatment strategies for early, late, chronic, and recurrent PTTO. We conclude that regardless of the etiology, a patient with persistent or recurrent PTTO should consider undergoing removal of the ventilation tube.

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.

Absent stapedial reflex: Otosclerosis or middle ear tumor?

February 25, 2013     Deb Biswas, FRCS-ORL, MS-ORL, MRCS, DOHNS; Ranjit K. Mal, FRCS
article

Abstract

We present an unusual case in which a patient diagnosed as having otosclerosis on the basis of clinical and audiologic findings actually had a middle ear facial nerve schwannoma. To the best of our knowledge, this is the first reported case in English literature in which a facial nerve schwannoma presented with conductive deafness of gradual onset and absent stapedial reflex with a normally functioning facial nerve. We also include a review of the literature.

Idiopathic incus necrosis: Analysis of 4 cases

February 25, 2013     Leyla Kansu, MD; Ismail Yilmaz, MD; Volkan Akdogan, MD; Suat Avci, MD; Levent Ozluoglu, MD
article

Abstract

We evaluated ossicular chain reconstruction in patients with idiopathic incus necrosis who have conductive hearing loss and an intact ear drum. The study included four patients (3 women and 1 man; the ages of the patients were 22, 31, 35, and 56 years, respectively) with unilateral conductive hearing loss, no history of chronic serous otitis media, an intact ear drum, normal middle ear mucosa, and necrosis of the long processes of the incus. On preoperative pure tone audiometry, air-bone gaps were 24, 25, 38, and 33 dB. Bilateral tympanometry and temporal bone computed tomography results were normal. All 4 patients underwent an exploratory tympanotomy. During the operation, the mucosa of the middle ear was normal, with a mobile stapes foot plate and malleus. No evidence of any granulation tissue was found; however, necrosis of the incus long processes was seen. For ossicular reconstruction, we used tragal cartilage between the incus and the stapes in 1 patient; in the other 3 patients, glass ionomer bone cement was used (an interposition cartilage graft also was used in the patients who received the glass ionomer bone cement). In all patients, air-bone gaps under 20 dB were established in the first year after surgery. In the ossicular disorders within the middle ear, the incus is the most commonly affected ossicle. While, the most common cause of these disorders is chronic otitis media, it may be idiopathic rarely. Several ossicular reconstruction techniques have been used to repair incudostapedial discontinuity.

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.

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