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Tufatulin G.Sh.

Mechnikov North-Western State Medical University;
Center of Pediatric audiology;
Saint Petersburg Scientific Research Institute of Ear, Throat, Nose and Speech;
Russian Medical Academy foR Continuous Professional Education;
Sverzhevsky Research Clinical Institute of Otorhinolaryngology

Khairulina S.I.

Center for Better Hearing

Kreisman M.V.

Mechnikov North-Western State Medical University;
City Clinical Policlinic No. 7

Kaloeva N.R.

Doctor Hearing LLC

Bakhshinyan V.V.

Mechnikov North-Western State Medical University;
Russian Medical Academy foR Continuous Professional Education

Koroleva I.V.

Mechnikov North-Western State Medical University;
Center of Pediatric audiology

Dvoryanchikov V.V.

Mechnikov North-Western State Medical University

Artyushkin S.A.

Mechnikov North-Western State Medical University;
Saint Petersburg Scientific Research Institute of Ear, Throat, Nose and Speech

Tavartkiladze G.A.

Russian Medical Academy foR Continuous Professional Education

Elevated ABR thresholds in the presence of otoacoustic emissions: case series

Authors:

Tufatulin G.Sh., Khairulina S.I., Kreisman M.V., Kaloeva N.R., Bakhshinyan V.V., Koroleva I.V., Dvoryanchikov V.V., Artyushkin S.A., Tavartkiladze G.A.

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Journal: Russian Bulletin of Otorhinolaryngology. 2026;91(1): 76‑85

DOI: 10.17116/otorino20269101176

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Table of contents

ELEVATED ABR THRESHOLDS IN THE PRESENCE OF OTOACOUSTIC EMISSIONS: CASE SERIES
ELEVATED ABR THRESHOLDS IN THE PRESENCE OF OTOACOUSTIC EMISSIONS: CASE SERIES

To cite this article:

Tufatulin GSh, Khairulina SI, Kreisman MV, et al. Elevated ABR thresholds in the presence of otoacoustic emissions: case series. Russian Bulletin of Otorhinolaryngology. 2026;91(1):76‑85. (In Russ.)
https://doi.org/10.17116/otorino20269101176

Подписка 2026 Вестник оториноларингологии (Russian Bulletin of Otorhinolaryngology)
МСФ на ЯМ
Использование инфографики авторами научных работ
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Abstract:

The article presents three clinical cases of an unusual audiological profile in children, not previously described in the literature. The cases are of interest due to the atypical symptomatology featuring preserved otoacoustic emissions (OAEs) concurrent with elevated thresholds of auditory brainstem responses (ABRs). Notably, wave V exhibited normal morphology and latency; in some instances, the ABR thresholds correlated with the behavioral thresholds. This clinical presentation cannot be classified as either conventional peripheral sensorineural hearing loss or an auditory neuropathy spectrum disorder. It is hypothesized that the morphological substrate of this condition is a lesion at the level of the inner hair cell or the synapse. The underlying pathogenesis is suggested to involve mild impairments in neural synchronization, which are sufficient to allow for the recording of ABRs with thresholds that, in some cases, correlate with behavioral responses.

Keywords:

auditory neuropathy
sensorineural hearing loss
auditory brainstem response
otoacoustic emissions

Authors:

Tufatulin G.Sh.

Mechnikov North-Western State Medical University;
Center of Pediatric audiology;
Saint Petersburg Scientific Research Institute of Ear, Throat, Nose and Speech;
Russian Medical Academy foR Continuous Professional Education;
Sverzhevsky Research Clinical Institute of Otorhinolaryngology

Khairulina S.I.

Center for Better Hearing

Kreisman M.V.

Mechnikov North-Western State Medical University;
City Clinical Policlinic No. 7

Kaloeva N.R.

Doctor Hearing LLC

Bakhshinyan V.V.

Mechnikov North-Western State Medical University;
Russian Medical Academy foR Continuous Professional Education

Koroleva I.V.

Mechnikov North-Western State Medical University;
Center of Pediatric audiology

Dvoryanchikov V.V.

Mechnikov North-Western State Medical University

Artyushkin S.A.

Mechnikov North-Western State Medical University;
Saint Petersburg Scientific Research Institute of Ear, Throat, Nose and Speech

Tavartkiladze G.A.

Russian Medical Academy foR Continuous Professional Education

Received:

22.08.2025

Accepted:

23.10.2025

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References:

  1. Tufatulin GSh, Lalayants MR, Artyushkin SA, Vikhnina SM, Garbaruk ES, Dvoryanchikov VV, Koroleva IV, Kreisman MV, Mefodovskaya EK, Pashkov AV, Savenko IV, Tsygankova ER, Chibisova SS, Tavartkiladze GA.Clinical protocol: audiological assessment of infants In Russian Federation. Part I. Bulletin of Otorhinolaryngology. 2023;88(5):82-90. (In Russ.). https://doi.org/10.17116/otorino20238805182
  2. Starr A, Picton TW, Sininger Y, Hood LJ, Berlin CI. Auditory neuropathy. Brain. 1996;119 (Pt 3):741-753.  https://doi.org/10.1093/brain/119.3.741
  3. Sharma A, Dorman MF, Spahr AJ. A sensitive period for the development of the central auditory system in children with cochlear implants: implications for age of implantation. Ear and Hearing. 2002;23(6):532-539.  https://doi.org/10.1097/00003446-200212000-00004
  4. Shim M, Yi J, Pak JH, Chung JW. Zinc deficiency triggers hearing loss by reducing ribbon synapses of inner hair cells in CBA/N mice. Biochemical and Biophysical Research Communications. 2024;693:149396. https://doi.org/10.1016/j.bbrc.2023.149396
  5. Safe TM, Luebke AE. Prenatal low dosage dioxin (TCDD) exposure impairs cochlear function resulting in auditory neuropathy. Hearing Research. 2016;331:7-12.  https://doi.org/10.1016/j.heares.2015.09.015
  6. Starr A, Sininger Y, Nguyen T, Michalewski HJ, Oba S, Abdala C. Cochlear receptor (microphonic and summating potentials, otoacoustic emissions) and auditory pathway (auditory brain stem potentials) activity in auditory neuropathy. Ear and Hearing. 2001;22(2):91-99.  https://doi.org/10.1097/00003446-200104000-00002
  7. Rance G, Beer DE, Cone-Wesson B, Shepherd RK, Dowell RC, King AM, Rickards FW, Clark GM. Clinical findings for a group of infants and young children with auditory neuropathy. Ear and Hearing. 1999;20:238-252.  https://doi.org/10.1097/00003446-199906000-00006
  8. Zeng FG, Oba S, Garde S, Sininger Y, Starr A. Temporal and speech processing deficits in auditory neuropathy. NeuroReport. 1999;10:3429-3435. https://doi.org/10.1097/00001756-199911080-00031
  9. British Society of Audiology. Recommended Procedure Assessment and Management of Auditory Neuropathy Spectrum Disorder (ANSD) in Young Infants. 2019. Accessed April 10, 2025. https://www.thebsa.org.uk/wp-content/uploads/2023/10/OD104-85-Recommended-Procedure-Assessment-and-Management-of-ANSD-in-Young-Infants.pdf.
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