Patricia M. Chute

A review of twenty congenital cholesteatomas of the middle ear in children.

Cholesteatomas, arising within the middle ear space behind an intact tympanic membrane, have been detected more frequently in recent years. This article reviews 19 children (with a mean age of 4.3 years) who underwent surgery over a 7-year period for removal of cholesteatomas from behind intact tympanic membranes. Most of these children were referred by pediatricians who had detected an asymptomatic whitish middle ear mass. Many had histories of treatment for otitis media. Patients with similar histories were excluded from the series if there had been a prior perforation, myringotomy, or otologic surgery. In the series, one fourth of the children had associated congenital malformations, and there were 3 times as many boys as girls. In two thirds of the cases, the cholesteatoma was manifested as an anterior-superior middle ear mass that seemed to arise from the processus cochleariformis, undersurface of malleus area. Many could be removed via an extended anterior tympanotomy operative approach. The results of surgery are reviewed. The possible origins of these localized cholesteatomas are considered. Are they congenital defects that arise from misplaced keratinizing epithelium? Do they arise from mesenchymal cells whose differentiation is stimulated by inflammatory changes within the middle ear?

Outcome Analysis of Cochlear Implant Reimplantation in Children

Objectives We review our e‐perience gained in performing cochlear reimplantation in 25 children who have had multichannel cochlear implant device failure at the Cochlear Implant Center of the Manhattan Eye, Ear and Throat Hospital and Lenox Hill Hospital (New York, NY), to assess the feasibility of cochlear reimplantation in children and the effect of reinsertion on audiological performance.

Specific language growth in young children using the CLARION® Cochlear Implant

General and specific language growth was evaluated in 10 children who had received the CLARION® Multi-Strategy™ Cochlear Implant during the clinical trial. The mean age at implantation was 37 months, and the children were followed for 18 months thereafter. Language comprehension and use were assessed with the Peabody Picture Vocabulary Test, the Reynell Developmental Language Scales, and mean length of utterance (MLU). The mean vocabulary scores indicated a 42-month increase in 18 months. The mean language comprehension increased from 20.4 to 40.8 months, and language use increased from 21.5 to 38.1 months. The mean MLU increased from 1.8 words to 4.8 words during the test period. Results indicated the children learned language at the same rate as their hearing peers of the same language age (except for vocabulary, which was faster), but retained a language delay relative to their hearing peers of the same chronological age.

Cholesteatoma in the Pediatric Age Group

The diagnosis and management of cholesteatoma in children remains controversial. In the past 15 years, the senior author (S.C.P.) has treated 320 patients with cholesteatoma. Patients 18 years and younger composed 40% (125) of the overall group and are the basis for this report. The patient data were compiled using the history, physical examination, audiograms, radiographs, patient questionnaires, surgical findings, and postoperative observations. The surgical treatment selected was determined by the extent of disease, the configuration of the mastoid, and a clinical assessment of eustachian tube function. A middle ear tympanotomy approach was used in 17% of the patients, a canal wall up procedure in 31 %, and a canal wall down procedure in 52.3 %. The average clinical follow-up was 3.9 years, with the range being from 3 months to 13.5 years. Hearing was maintained or slightly improved in a majority of cases. Residual disease occurred in 8 % of patients, and recurrent disease in only 3 %.

The relationship between electrical acoustic reflex thresholds and behavioral comfort levels in children and adult cochlear implant patients.

The accuracy with which behavioral comfort levels could be predicted by the electrically elicited acoustic reflex threshold (EART) was examined in 35 Nucleus Cochlear Implant patients (16 adults and 19 children). EARTs were obtained by stimulating bipolar pairs of electrodes through the Nucleus Diagnostic Programming System and monitoring the change in middle ear admittance in the ear contralateral to the implanted ear. EARTs were successfully elicited in 24 patients. EARTs differed from behavioral comfort levels by a mean of 19.4 stimulus level units for adults and 9.6 stimulus level units for children. While EARTs were found to be acceptably close to behavioral comfort levels in four adults and eight children, EARTs significantly overestimated or underestimated comfort levels in the rest. The results of this study suggested that while the EART does not accurately predict comfort levels in all cases, it may provide valuable information regarding levels which should not be exceeded when programming the cochlear implant. Cautious use of information available from the EART may prove useful for programming the cochlear implant in children or adults who are unable to make reliable psychophysical judgments.

Cochlear Implant Flap Complications

In a series of 52 patients who received cochlear implants, 4 patients suffered flap complications (7.7%). The problems encountered involved the postauricular flap and were usually minor in nature. None required explantation as a direct result of these complications. Flap ischemia in a patient with Cogans syndrome and vasculitis, two cases of suture extrusion with one having exposure of the implant, and a case of receiver unit magnet extrusion repaired with a vascularized pericranial flap based upon temporalis muscle are presented. Flap design in patients who have had postauricular incisions demands special consideration. Principles useful for avoiding complications as well as their management are discussed.

Speech changes following reimplantation from a single‐channel to a multichannel cochlear implant

The speech of a postlingually deafened preadolescent was recorded and analyzed while a single-electrode cochlear implant (3M/House) was in operation, on two occasions after it failed (1 day and 18 days) and on three occasions after stimulation of a multichannel cochlear implant (Nucleus 22) (1 day, 6 months, and 1 year). Listeners judged 3M/House tokens to be the most normal until the subject had one years experience with the Nucleus device. Spectrograms showed less aspiration, better formant definition and longer final frication and closure duration post-Nucleus stimulation (6 MO. NUCLEUS and 1 YEAR NUCLEUS) relative to the 3M/House and no auditory feedback conditions. Acoustic measurements after loss of auditory feedback (1 DAY FAIL and 18 DAYS FAIL) indicated a constriction of vowel space. Appropriately higher fundamental frequency for stressed than unstressed syllables, an expansion of vowel space and improvement in some aspects of production of voicing, manner and place of articulation were noted one year post-Nucleus stimulation. Loss of auditory feedback results are related to the literature on the effects of postlingual deafness on speech. Nucleus and 3M/House effects on speech are discussed in terms of speech production studies of single-electrode and multichannel patients.

Classroom performance of children with cochlear implants in mainstream education.

Objectives: We compared classroom performance of children with a cochlear implant (CI) with that of their normal-hearing peers in mainstream education. Methods: Thirty-two CI children in mainstream education, congenitally or prelingually deaf, participated in this study, as did 37 hearing classmates. Their teachers filled out 2 questionnaires: the Assessment of Mainstream Performance (AMP) and the Screening Instrument for Targeting Educational Risk (SIFTER). A high Fletcher index and open-set speech perception scores were obtained. Results: The children with CIs scored above average on the AMP and sufficiently well in all but one area (communication) of the SIFTER questionnaire. Class rankings did not differ significantly between the CI students and their normal-hearing peers. Overall, the normal-hearing group outperformed the CI group. The classroom performance of CI children correlated negatively with duration of deafness and age at implantation. All longitudinal audiological data of the CI children showed improvement in open-set speech recognition. Conclusions: Although the results are encouraging, the CI group scored significantly less well than their normal-hearing peers on most questionnaire domains of both the AMP and the SIFTER. The most important variables for the outcome in this study were age at implantation and duration of deafness.

Educational Challenges for Children with Cochlear Implants

Children with severe to profound hearing loss who receive cochlear implants have an opportunity to develop the auditory skills that will assist them in lifelong learning. Although the auditory access provided by a cochlear implant can make this process easier, there remain a number of challenges tha

The speech of a postlingually deafened teenager during the first year of use of a multichannel cochlear implant

The speech of a profoundly postlingually deafened teenager was recorded before, immediately after, 3 months after, and 1 year after electrical stimulation with a Nucleus multichannel cochlear implant. Listener tests of target words revealed significant improvement in overall quality over the year. Spectrograms showed less aspiration and better definition of the lower formants. Acoustic measurements indicated immediate change in F0 and gradual changes in syllable duration and some aspects of voicing and manner of articulation. Vowel space shrank steadily over the year, with both first- and second-formant frequencies dropping. Prestimulation results are discussed relative to the literature on the speech of the congenitally hearing impaired. Effects of multichannel electrical stimulation on speech are compared with studies of single-electrode stimulation.