We reviewed recent progress in otoacoustic emission (OAE) as an objective hearing test tool. OAE features the advantage of objective, noninvasive measurement of the ear's ability to process acoustic stimuli. Since its discovery in 1978, numerous clinical applications have been established, including their utility in screening cochlear function in infants and other difficult-to-test patients, in the monitoring of outer hair cell function in patients exposed to potentially damaging agents or who have progressive hearing loss, and in the differential diagnosis of sensorineural hearing loss. OAE has thus become the standard tool for hearing screening in pediatric patients.
Maternal anxiety has been considered as a possible disadvantage of Newborn Hearing Screening (NHS). We reviewed the results of an interview survey conducted by public-health nurses (PHN) during initial home visits performed as part of the Okayama Prefecture NHS Program. The questionnaires assessed maternal anxiety, worry, certainty about the babies' hearing, and maternal attitude toward hearing screening. In total, 47, 346 newborns underwent NHS in Okayama Prefecture, and 248 babies were referred for further evaluation, One hundred seventy-one interviews were available for review in this study. Thirtynine (22.8%) mothers complained of anxiety or worry during the home visit by the PHN. Nineteen cases (11.1%) were diagnosed as having additional handicaps, and the major causes of maternal anxiety in these cases were severer disabilities other than hearing impairment. Sixteen cases (9.4%) were in the process of coping with a confirmed or potential diagnosis of hearing impairment. Only 4 cases (2.3%) demonstrated unexpected anxiety because of a suspicion of hemilateral hearing impairment. Child-neglect was observed in one case with multiple anomalies. Although the incidence of maternal anxiety after NHS was not high, compared with maternal anxiety in the absence of NHS, efforts to minimize maternal anxiety and worry, especially of mothers whose infants are referred for further evaluation, will be indispensable to establishing workable NHS programs in Japan.
The development of hearing in newborn infants with hearing loss critically depends on education after a diagnosis has been made. Using a hearing ability scoring system, we evaluated the development of hearing in 11 patients (including 6 patients with multiple handicaps) who were diagnosed with hearing impairment by a neonatal hearing screening test between 2002 and 2003. The score of hearing development was lower for children with hearing impairment and multiple disabilities than for children who only had hearing impairment. However, proper educational programs and hearing aids benefited hearing development in children with multiple handicaps. In the 6 children with multiple disabilities, hearing development in patients with Down syndrome or congenital cytomegaloviral infection was faster than that in patients with auditory neuropathy. Since some newborn infants with hearing loss show an unpredictable fluctuation in hearing even one year after birth or the development of communication disorders like infantile autism, educational programs and hearing aids appropriate to the physical and mental conditions of the patients are critical.
In recent years, Tinnitus Retraining Therapy (TRT) has become a popular treatment for tinnitus throughout the world. We have used TRT (with a sound generator) to treat 56 patients (26 men, 30 women) at the Department of Otolaryngology, Nagoya City University hospital. Thirty-two cases were evaluated using the tinnitus handicap inventory (THI) and a visual analogue scale (VAS: awareness, loudness, and duration of tinnitus) at 12 months after onset. The THI scores improved in 88% of the patients, and the VAS (awareness) scores improved in 76%. TRT had significantly improved the VAS (awareness) and THI scores after 3 months. The VAS (loudness) scores improved after 6 months. Sixteen cases were followed for 18-24 months, and TRT improved the THI scores in these patients.
The language skills of 12 deaf children with cochlear implants (CI group) were compared with those of 19 children with conventional hearing aids (HA group) after due consideration of language foundation (LF). The HA group was divided into three types of children, according to the characteristics of their language progress: rapid, consisting of children with well-developed LF; delayed, consisting of children with poorly developed LF; and middle. The language skills of the CI group before cochlear implantation were similar to those of the delayed group, but their language skills developed rapidly after cochlear implantation. The earlier the devices were implanted, the faster the children's language skills developed. All the HA children in the delayed group had a poor LF, whereas the CI group included only one child (Case S) with poor LF. S's language before implantation had been delayed, but after the operation his language skills gradually improved and were within the normal range by his 5th year.
Sweets' syndrome is an usual disease characterized by fever, leukocytosis, and distinctive skin lesions. The involvement of the central nervous system in Sweets' syndrome is termed neuro-Sweet disease. Only a few reports have mentioned sensorineural hearing loss in neuro-Sweet disease. Here, we present a case of neuro-Sweet disease with sensorineural hearing loss. A 33-year-old man experienced bilateral recurrent acute sensorineural hearing loss with meningitis and encephalitis. Magnetic resonance images showed increased signal intensity in the white matter on T 2-weighted and FLAIR scans. HLA typing revealed B54 and CW1, which are frequently observed in Sweets' syndrome. Steroid therapy was remarkably effective for alleviating the sensorineural hearing loss as well as the meningitis and encephalitis. Analysis of an SISI test, Bekesy test, auditory brain stem response and SPELT abnormality revealed both cochlear and brainstem lesions. Neuro-Sweet disease should be included in a differential diagnosis of unexplainable recurrent acute sensorineural hearing loss complicated by meningitis and encephalitis.
We studied 53 children with mild, moderate or moderately severe hearing impairments who visited the National Mie Hospital. The following results were obtained. 1) Seven children had syndromic hearing impairments, 44 had nonsyndromic hearing impairments, and two children had a high-risk of hearing impairment. 2) Patient age ranged from one month to fifteen years old (mean, five years and six months), and their average hearing ranged from 25.0dBHL to 69.9dBHL. Two peaks in patient age were detected: one at under 6 months of age, and the other at 6 years of age. 3) Four of the seven infants whose hearing impairments were detected before the age of 6 months were identified during newborn hearing examinations. 4) Thirty-one infants or children were fitted with hearing aids. Hearing aid use and the amount of time that the hearing aids were used for were related to the hearing levels of the patients. 5) Seven children with bilateral sensorineural hearing loss of more than 40dB without the use of hearing aids were diagnosed at an age of 4 years or older, and five of these patients exhibited language problems. Recently, it has become possible to detect hearing impairments in children at an earlier age, subsequent guidance and intervention by specialists are needed.
Communicating with hearing impaired children depends not only on the aural communication ability of the child, but of the conversation partner. In this study, we analyzed four different telephone conversations held with a 12-year-old boy with a profound hearing impairment. The conversations were between the boy and a Telephone Communication Training (TCT) teacher, his mother, and two female teachers (A and B) who teach physically and mentally handicapped children. The boy had received TCT. The manner and content of the aural communication between the boy and each speaker was studied. In addition, the rate of appropriate responses from the boy was recorded. The speech of the TCT teacher appeared to be the easiest for the boy to understand, while that of teacher B was the most difficult. The TCT teacher's sentences consisted of four words or less. She intentionally made frequent use of communication strategy skills and avoided using words of agreement. On the other hand, the speech of teacher B was passive, and the teacher used long sentences and employed fewer strategy skills. In addition, the aural communication style of the child was also clearly affected by the conversational partner's manner of speaking. When what was said cannot be understood, communication strategy skills include asking again (Pardon?)”, requesting information (Could you speak slowly?), and reconfirmation. It is important to use short sentences and communication strategy skills when communicating with hearing impaired children, and not simply ignore the child; this encourages the child to talk. By using many strategy skills, comprehensible conversation can be held. However, language training, such as TCT, appears to be necessary to develop the communication ability of hearing impaired children in both bottom-up processes, like hearing and pronunciation, as well as top-down processes, like thinking.
This study investigated the ability of children with hearing impairments to identify prosodic information. Ten children with hearing impairments received pitch identification tests using syllable accents (“ame”: increasing accent=“candy”, decreasing accent=“rain”) and sentence intonation (statement and question) as test materials. The materials consisted of six synthetic speech stimuli with different F0 values for the two accent types. Time expansion and pitch increase were added as further variables. The subjects were instructed to identify the pitch accent and to choose between two pictures of the test materials. The results were then compared with those for normal children. Seven of the ten children with hearing impairments were able to identify prosodic information either perfectly or imperfectly. Children with slight hearing impairments scored better than those with serious hearing impairments. The children's abilities to identify prosodic information were related to their auditory thresholds. 3) The ability to identify prosodic information was influenced by the degree of auditory modality use. 4) The results of the syllable accent and sentence intonation tests corresponded in 4 of the 10 children; both assessments were influenced by individual differences.
Sarcoidosis is a systemic granulomatous disease of unknown etiology. We report a 27-year-old woman with systemic sarcoidosis and bilateral sensorineural hearing loss (SNHL). Other findings included granulomatous uveitis, dysfunction of the bilateral fifth cranial nerves, and hilar adenopathies. She first suffered from right sudden hearing loss and consulted an otolaryngologist. A pure-tone audiogram revealed a right highfrequency SNHL that spontaneously recovered. One month later, she presented at our department with left sudden hearing loss, tinnitus, and aural fullness. A pure-tone audiogram revealed a left Ushaped SNHL and a right mild SNHL. Further audiological examinations including speech audiometry, self-recording audiometry, distortionproduct otoacoustic emissions, electrocochleography, and auditory brainstem responses revealed cochlear dysfunction with a cochlear nerve conduction block. Steroid therapy for a 10-day period was insufficient, and recovery from the left SNHL was incomplete. The patient was diagnosed as having systemic sarcoidosis based on pulmonological and serological studies. During the course of her illness, her left SNHL normalized four months after onset. Although transient right SNHL occurred, the patient's normal hearing persisted. The clinical course of her hearing loss could not entirely refute a diagnosis of endolymphatic hydrops.