The Kresge Hearing Research Institute-3 (KHRI-3) antibody binds to a guinea pig inner ear supporting cell antigen (IESCA) and causes hearing loss. To gain insight into the mechanism of antibody-induced hearing loss, we used antibody immunoaffinity purification to isolate the IESCA, which was then sequenced by mass spectroscopy, revealing 10 guinea pig peptides identical to sequences in human choline transporter-like protein 2 (CTL2). Full-length CTL2 cDNA sequenced from guinea pig inner ear has 85.9% identity with the human cDNA. Consistent with its expression on the surface of supporting cells in the inner ear, CTL2 contains 10 predicted membrane-spanning regions with multiple N-glycosylation sites. The 68 and 72 kDa molecular forms of inner ear CTL2 are distinguished by sialic acid modification of the carbohydrate. The KHRI-3 antibody binds to an N-linked carbohydrate on CTL2 and presumably damages the organ of Corti by blocking the transporter function of this molecule. CTL2 mRNA and protein are abundantly expressed in human inner ear. Sera from patients with autoimmune hearing loss bind to guinea pig inner ear with the same pattern as CTL2 antibodies. Thus, CTL2 is a possible target of autoimmune hearing loss in humans.

Higher levels of cytokines in patients with cholesteatoma confirm that the destructive behavior of cholesteatoma is likely mediated by cytokines and epidermal growth factor and is the result of keratinocyte activity. Antibiotic treatment does not affect the level of cytokine concentration in patients with chronic otitis media and cholesteatoma, although the ear discharge subsides and inflammation-related symptoms regress in some cases.

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Surgeon and patient alike would always choose a hearing preservation technique if there was no potential for increased morbidity in making the attempt. When compared with the non-hearing preservation translabyrinthine approach, the retrosigmoid approach had a higher incidence of persistent headache. In addition, efforts to conserve the auditory nerve prolong operating time, increase the incidence of postoperative vestibular dysfunction, and carry a slightly higher risk of tumor recurrence. Nevertheless, even though the probability of success is disappointingly small, when excellent hearing is present we favor offering the option of a hearing conservation attempt when the patient has been well informed of the pros and cons of the endeavor. Factors weighing against undertaking this effort include larger cerebellopontine angle component (>or=25 mm), deep involvement of the fundus, wide erosion of the porus, and marginal residual hearing.

Syndromic and nonsyndromic cases of isolated semicircular canal aplasia were identified. Except for mild to moderate cochlear dysplasia, and the anomalous course of the facial nerve in some CHARGE association patients, both groups of patients were generally suitable for cochlear implantation if indicated. A high incidence of oval window aplasia with normal round window development may help to explain the embryopathogenesis of this anomaly, considering the sequence of inner ear development.