TITLE

Audiological and electrocochleography findings in hearing-impaired children with connexin 26 mutations and otoacoustic emissions

AUTHOR(S)
Santarelli, Rosamaria; Cama, Elona; Scimemi, Pietro; Dal Monte, Erica; Genovese, Elisabetta; Arslan, Edoardo
PUB. DATE
January 2008
SOURCE
European Archives of Oto-Rhino-Laryngology;Jan2008, Vol. 265 Issue 1, p43
SOURCE TYPE
Academic Journal
DOC. TYPE
Article
ABSTRACT
We recorded cochlear potentials by transtympanic electrocochleography (ECochG) in three hearing-impaired children with GJB2 mutation who showed otoacoustic emissions. Pure tone thresholds, distortion product otoacoustic emissions (DPOAEs) and, auditory brainstem responses (ABRs) were also obtained. Subjects 1 (35delG/35delG) and 3 (M34T/wt) had profound hearing loss and showed the picture of auditory neuropathy (AN) as DPOAEs were detected with absent ABRs in both ears. The hearing impairment found in subject 2 (35delG/35delG) was profound in the right ear and moderate in the left ear. Both DPOAEs and ABRs with normal latencies and morphology were recorded only from the left ear. On the ECochG recording the cochlear microphonic was obtained from all children. No compound action potential (CAP) was detected in subject 1. A neural response was recorded only from the left ear in subject 2 with a threshold corresponding to the audiometric threshold while no CAP was detected on the right side. The ECochG obtained from subject 3 showed a low-amplitude broad negative deflection which was identifiable down to low stimulus levels. This response decreased in amplitude and duration when utilizing a high-rate stimulation paradigm. The amount of amplitude reduction was close to that calculated for normal ears, thus revealing the presence of an adapting neural component. These findings indicate that patients with GJB2 mutations and preserved outer hair cells function could present with the picture of AN. The hearing impairment is underlain by a selective inner hair cell loss or a lesion involving the synapses and/or the auditory nerve terminals. We suggest that neonatal hyperbilirubinemia may play a role in protecting outer hair cells against the damage induced by GJB2 mutations.
ACCESSION #
27525836

 

Related Articles

  • CONSIDERACIONES EN LA AMPLIFICACIÓN DE NIÑOS CON HIPOACUSIA MÍNIMA O LEVE Y PÉRDIDA AUDITIVA UNILATERAL.  // Revista Chilena de Tecnología Médica;2012, Vol. 32 Issue 2, p1732 

    No abstract available.

  • DPOAE Changes in Young Children With Confirmed Hearing Loss Due to Ototoxicity. Beahan, Nuala; Reichman, Emma; Kei, Joseph; Driscoll, Carlie; Young, June; Suppiah, Ram; Grohn, Mary-Lou; Sockalingam, Ravi; Charles, Bruce // Australian & New Zealand Journal of Audiology;Nov2006, Vol. 28 Issue 2, p90 

    The aim of this study was to examine the pattern of changes in distortion product otoacoustic emissions (DPOAEs) in children with ototoxic hearing loss during chemotherapy. The participants included a control group of 15 normal hearing children (3–12 years) and an experimental group of 7...

  • A Multisite Study to Examine the Efficacy of the Otoacoustic Emission/Automated Auditory Brainstem Response Newborn Hearing Screening Protocol: Recommendations for Policy, Practice, and Research. Gravel, Judith S.; White, Karl R.; Johnson, Jean L.; Widen, Judith E.; Vohr, Betty R.; James, Michele; Kennalley, Teresa; Maxon, Antonia B.; Spivak, Lynn; Sullivan-Mahoney, Maureen; Weirather, Yusnita; Meyer, Sally // American Journal of Audiology;Dec2005, Vol. 14 Issue 2, pS217 

    Purpose: This article examines whether changes in hearing screening practices are warranted based on the results of the recent series of studies by J. L. Johnson, K. R. White, J. E. Widen, J. S. Gravel, B. R. Vohr, M. James, T. Kennalley, A. B. Maxon, L. Spivak, M. Sullivan-Mahoney, Y....

  • A Multisite Study to Examine the Efficacy of the Otoacoustic Emission/Automated Auditory Brainstem Response Newborn Hearing Screening Protocol: Results of Visual Reinforcement Audiometry. Widen, Judith E.; Johnson, Jean L.; White, Karl R.; Gravel, Judith S.; Vohr, Betty R.; James, Michele; Kennalley, Teresa; Maxon, Antonia B.; Spivak, Lynn; Sullivan-Mahoney, Maureen; Weirather, Yusnita; Meyer, Sally // American Journal of Audiology;Dec2005, Vol. 14 Issue 2, pS200 

    Purpose: This 3rd of 4 articles on a study of the efficacy of the 2-stage otoacoustic emission/automated auditory brainstem response (OAE/A-ABR) newborn hearing screening protocol describes (a) the behavioral audiometric protocol used to validate hearing status at 8-12 months of age, (b) the...

  • Results of a newborn hearing screening program in Cuiabá - Mato Grosso, Brazil. de Araújo Lucas Rodrigues, Priscila; de Carvalho, Thiago Serra Ferreira; Pereira Lauris, José Roberto; Schochat, Eliane // Revista da Sociedade Brasileira de Fonoaudiologia;2011, Vol. 16 Issue 4, p454 

    Purpose: To describe the results obtained in a newborn hearing screening program in the city of Cuiabá (MT), Brazil, in the period from 2009 to 2010. Methods: We analyzed the otoacoustic emissions results of 1964 low-risk and 123 high-risk newborns, archived in the computer of the service,...

  • NEWBORN HEARING SCREENING: CHARACTERIZATION OF DEMAND/TERRITORY AND HEARING TESTS. Hernandez Rogério, Amanda Ferreira; Teixeira Amado, Bárbara Carolina; Crepaldi de Almeida, Elizabeth Oliveira; Umeoka Hidaka, MarieneTerumi // Revista CEFAC;jul/ago2014, Vol. 16 Issue 4, p1060 

    Purpose: to characterize the demand, territory and hearing tests performed on a Newborn Hearing Screening Program. Methods: retrospective study of a sample of 2334 records of newborns screened, involving the analysis of data on Newborn Hearing Screening, Newborns of information and demographic...

  • NEWBORN HEARING SCREENING: CHARACTERIZATION OF DEMAND/TERRITORY AND HEARING TESTS. Hernandez Rogério, Amanda Ferreira; Teixeira Amado, Bárbara Carolina; Crepaldi de Almeida, Elizabeth Oliveira; MarieneTerumi Umeoka Hidaka // Revista CEFAC;jul/ago2014, Vol. 16 Issue 4, p1069 

    Purpose: to characterize the demand, territory and hearing tests performed on a Newborn Hearing Screening Program. Methods: retrospective study of a sample of 2334 records of newborns screened, involving the analysis of data on Newborn Hearing Screening, Newborns of information and demographic...

  • Community-based infant hearing screening in a developing country: parental uptake of follow-up services. Olusanya, Bolajoko O.; Akinyemi, Oladele O. // BMC Public Health;2009, Vol. 9 Issue 1, p1 

    Background: Universal newborn hearing screening is now considered an essential public health care for the early detection of disabling life-long childhood hearing impairment globally. However, like any health interventions in early childhood, parental support and participation is essential for...

  • Time-Frequency Analysis of Transient-Evoked Otoacoustic Emissions in Children Exposed to Carboplatin Chemotherapy. Bhagat, Shaum; Bass, Johnnie; Qaddoumi, Ibrahim; Brennan, Rachel; Wilson, Matthew; Wu, Jianrong; Galindo, Carlos-Rodriguez; Paglialonga, alessia; Tognola, Gabriella // Audiology & Neuro-Otology;Jan2013, Vol. 18 Issue 2, p71 

    The aims of this study were to characterize and quantify time-frequency changes in transient-evoked otoacoustic emissions (TEOAEs) recorded in children diagnosed with retinoblastoma who were receiving carboplatin chemotherapy. A signal processing technique, the wavelet transform (WT), was used...

Share

Read the Article

Courtesy of THE LIBRARY OF VIRGINIA

Sorry, but this item is not currently available from your library.

Try another library?
Sign out of this library

Other Topics