February 25, 2013 Deb Biswas, FRCS-ORL, MS-ORL, MRCS, DOHNS; Ranjit K. Mal, FRCS
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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.
February 25, 2013 Willis S.S. Tsang, FRCSEd(ORL); Michael C.F. Tong, MD(CUHK); C. Andrew van Hasselt, M Med(Otol)
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Extrusion of ossicular prostheses results from postoperative tympanic membrane medialization. The incorporation of cartilage grafting material is recommended to minimize extrusion.
January 24, 2013 Leonardo Manzari, MD; Paola Scagnelli, MD
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Abstract
Superior semicircular canal dehiscence and an abnormally wide internal auditory meatus are clinical entities characterized by vestibular and cochlear symptoms. These symptoms are induced by hypersensitivity of labyrinthine receptors secondary to a bone defect of the otic capsule. We report the case of a 41-year-old man with congenital right-sided hearing loss who presented with bilateral superior semicircular canal dehiscence that was associated with wide, bulbous internal auditory meatus and a loss of the bony wall separating the lateral end of the meatus from the cochlea. The patient was experiencing vestibular and cochlear symptoms in the right ear and disabling tinnitus in the left ear. However, he refused all treatment and was lost to follow-up.
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
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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.
January 24, 2013 Tripti Brar, MBBS, MS, DNB; Sumit Mrig, MBBS, MS, DNB; J.C. Passey, MS; A.K. Agarwal, MS; Shayma Jain, MD
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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.
January 24, 2013 Aroor Rajeshwary, MS (ENT); Gangadhara Somayaji, MS (ENT); Sai Manohar, MS (ENT)
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Abstract
A 4-year-old boy presented with a painless swelling over the right pinna of 4 months' duration. Histopathologic examination of the excised mass revealed features of pilomatricoma-a rare, benign skin neoplasm arising from hair follicle matrix cells. The unusual location of the lesion over the pinna, despite its frequent occurrence in the head and neck, prompted this article. We discuss the etiology, clinical presentation, and management of pilomatricoma of the auricular region
January 24, 2013 Joseph A. Ursick, MD; Jose N. Fayad, MD
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Exostoses, composed of immature layers of lamellar bone that cause a progressive stenosis of the external ear canal, are typically bilateral, occur at suture lines, and are associated with repeated exposure to cold water.
January 24, 2013 Li-Tai Tsai, MD; Ching-Yuan Wang, MD; Chia-Der Lin, MD; Ming-Hsui Tsai, MD
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Abstract
Nontuberculous mycobacterial otomastoiditis is rare and can be easily confused with various different forms of otitis media. We describe the case of a 50-year-old woman who presented with left-sided chronic otitis media that had persisted for more than 1 year. It was not eradicated by standard antimicrobial therapy and surgical debridement. After appropriate antibiotic therapy for nontuberculous mycobacteria was added to the therapeutic regimen, the patient improved significantly and the lesion had healed by 6 months. Based on our experience with this case, we conclude that early bacterial culture and staining for acid-fast bacilli in ear drainage material or granulation tissue should be performed when standard antimicrobial therapy fails to eradicate chronic otitis media of an undetermined origin that is accompanied by granulation tissue over the external auditory canal or middle ear. Polymerase chain reaction testing is also effective for rapid diagnosis. Surgical debridement and removal of the foreign body can successfully treat nontuberculous mycobacterial otomastoiditis only when effective antimicrobial therapy is also administered.
December 31, 2012 Waitsz T. Chang, MRCS; Willis S.S. Tsang, FRCSEd(ORL)
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For the treatment of a small glomus tympanicum, surgical excision via an anterior tympanotomy approach is possible.
December 31, 2012 Nuno Marcal, MD; Sofia Campelos, MD; Luis Dias, MD; Matos Goncalves, MD; Gabriel Pereira, MD; Tiago Godinho, MD
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Abstract
Primary cutaneous T-cell lymphoma is rare. Cutaneous lymphoma is defined as primary when there is an absence of nodal or systemic disease during the first 6 months following diagnosis. We report what we believe to be the first documented case of a primary cutaneous CD30-positive anaplastic large-cell lymphoma of the external auditory canal. The patient was an elderly woman who presented with progressively worsening right otalgia and hypoacusis. Otoscopy revealed an erythematic, ulcerative, nonbleeding, localized lesion in the anterosuperior area of the external auditory canal. The patient underwent an excisional biopsy, and after the diagnosis was established, she underwent 22 sessions of radiotherapy. During follow-up, she exhibited no evidence of recurrence.
December 31, 2012 Ryan M. Rehl, MD; Sepehr Oliaei, MD; Kasra Ziai, MD; Hossein Mahboubi, MD, MPH; Hamid R. Djalilian, MD
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Abstract
A cadaveric study was performed to test the hypothesis that intact-canal-wall mastoidectomy (ICWM) with otoendoscopy allows for equal or better visualization of the middle ear cavity structures when compared with canal-wall-down mastoidectomy (CWDM) with microscopy. Ten temporal bones were prepared with a reversible canal-wall-down tympanomastoidectomy technique. Five anatomic sites in each middle ear cavity (lateral epitympanum, posterior crus of the stapes, the sinus tympani, eustachian tube orifice, and round window niche) were marked with paint. Two otolaryngologists blinded to the purpose of the study viewed the temporal bones with the microscope. Following replacement of the posterior canal walls, the bones were then viewed with a 30° and a 70° otoendoscope. All visualized paint marks for each viewing were recorded and compared. We found that ICWM with 30° or 70° otoendoscopy provided significantly better visualization of the sinus tympani than did CWDM (p ≤ 0.001). There was no significant difference among the three methods in visualization of the lateral epitympanum, posterior crus of the stapes, and round window niche. With respect to the eustachian tube orifice, one of the observers reported significantly better visualization with CWDM (p = 0.036). With adjunctive otoendoscopy, it is not necessary to remove the posterior canal wall to adequately visualize or remove disease from various areas of the middle ear cleft. The use of otoendoscopy during cholesteatoma surgery may allow for more frequent preservation of the posterior canal wall and reduced rates of residual cholesteatoma, given the equal or better visualization of the middle ear cavity.
December 31, 2012 LT Philip Gaudreau, MD; LCDR Justin Moy, MD; Fred Lindsay, DO
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Abstract
We describe the case of a 36-year-old woman with a history of vitiligo who presented with an insidious onset of neurologic, vestibular, ocular, and auditory symptoms. She had recently noted the onset of vertigo, tinnitus, and hypersensitivity to sound. Findings on audiometry were within normal limits, although the patient reported some auditory discomfort during the testing. The patient had a history of bilateral uveitis and peripheral neurologic symptoms. She was diagnosed with Vogt-Koyanagi-Harada (VKH) syndrome and started on corticosteroid therapy. Her neurologic, vestibular, ocular, and auditory symptoms resolved. VKH syndrome is an uncommon cause of vertigo and hearing loss, but it should be considered in the differential diagnosis of patients with autoimmunity-related inner ear symptoms.
