This study examines the relationship between the teaching method, oral or total communication, used at children's schools and children's consonant-production accuracy and vocabulary development over time. Children who participated in the study (N = 147) demonstrated profound sensorineural hearing loss and had used cochlear implants for between 6 months and 10 years. Educational programs that used an oral communication (OC) approach focused on the development of spoken language, whereas educational programs that used a total communication (TC) approach focused on the development of language using both signed and spoken language. Using Hierarchical Linear Modeling (HLM) we compared the consonant-production accuracy, receptive spoken vocabulary, and expressive spoken and/or signed vocabulary skills, over time, of children who were enrolled in schools that used either OC or TC approaches, while controlling for a number of variables. These variables included age at implantation, preoperative aided speech detection thresholds, type of cochlear implant device used, and whether a complete or incomplete active electrode array was implanted. The results of this study indicated that as they used their implants the children demonstrated improved consonant-production accuracy and expressive and receptive vocabulary over time, regardless of whether their school employed a TC or OC teaching method. Furthermore, there appeared to be a complex relationship among children's performance with the cochlear implant, age at implantation, and communication/teaching strategy employed by the school. Controlling for all variables, children in OC programs demonstrated, on average, superior consonant-production accuracy, with significantly greater rates of improvement in consonant-production accuracy scores over time compared to children in TC programs. However, there was no significant difference between OC and TC groups in performance or rate of growth in consonant-production accuracy when children received their implants before the age of 5 years. There was no significant difference between the OC and TC groups in receptive spoken vocabulary scores or in rate of improvement over time. However, children in the TC group achieved significantly higher receptive spoken vocabulary scores than children in the OC group if they received their implant before the age of 5 years. The TC group demonstrated superior scores and rates of growth on the expressive vocabulary measure (spoken and/or signed) when compared to the OC group if they received their implants during their preschool or early elementary school years. There was no significant difference if the children received their implants during middle elementary school. Regardless of whether children were in the OC or TC group, children who received their implants during preschool demonstrated stronger performance, on average, on all measures over time than children who received their implants during their elementary school years. The results of this study suggest that children may benefit from us...

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.

The BAHA bone-anchored hearing aid provides a reliable and predictable adjunct for auditory rehabilitation in appropriately selected patients, offering a means of dramatically improving hearing thresholds in patients with conductive or mixed hearing loss who are otherwise unable to benefit from traditional hearing aids.

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These results are in agreement with those of previous studies indicating that early implantation facilitates improved development of speech perception skills in profoundly deaf children.

By achieving excellent exposure and using meticulous microsurgical technique, it is possible to resect small vestibular schwannomas via the middle fossa approach, with preservation of hearing at excellent or preoperative levels in the majority of patients, with excellent or satisfactory facial nerve outcomes in 96% of patients.

The large vestibular aqueduct syndrome (LVAS) is a distinct clinical entity characterized by stepwise progressive sensorineural hearing loss associated with isolated enlargement of the vestibular aqueduct. A correlative clinical, audiologic, vestibular, cytogenetic, and radiographic analysis of a family with inherited LVAS was performed. The male proband and his affected brother are offspring of unaffected parents, and have no other abnormalities. Pedigree analysis suggests autosomal recessive or X-linked inheritance with variable expressivity of LVAS in this family. This study is the first description of familial inheritance of LVAS. LVAS may account for a significant number of patients with nonsyndromal, genetic sensorineural hearing loss. Future molecular analyses of this study family may identify the causative gene(s) in LVAS.

Children with radiographic cochlear malformations benefit from cochlear implantation with multichannel devices. They ultimately perform as well as their matched counterparts with normal cochleae, although they may improve more slowly over time.