A Prospective Study of Etiology and Auditory Profiles in Infants with Congenital Unilateral Sensorineural Hearing Loss
Congenital unilateral sensorineural hearing loss (uSNHL) is associated with speech-language delays and academic difficulties. Yet, controversy exists in the choice of diagnosis and intervention methods. A cross-sectional prospective design was used to study hearing loss cause in twenty infants with...
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| Format: | Article |
| Language: | English |
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MDPI AG
2022-07-01
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| Series: | Journal of Clinical Medicine |
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| Online Access: | https://www.mdpi.com/2077-0383/11/14/3966 |
| _version_ | 1797406052073865216 |
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| author | Marlin Johansson Eva Karltorp Kaijsa Edholm Maria Drott Erik Berninger |
| author_facet | Marlin Johansson Eva Karltorp Kaijsa Edholm Maria Drott Erik Berninger |
| author_sort | Marlin Johansson |
| collection | DOAJ |
| description | Congenital unilateral sensorineural hearing loss (uSNHL) is associated with speech-language delays and academic difficulties. Yet, controversy exists in the choice of diagnosis and intervention methods. A cross-sectional prospective design was used to study hearing loss cause in twenty infants with congenital uSNHL consecutively recruited from a universal neonatal hearing-screening program. All normal-hearing ears showed ≤20 dB nHL auditory brainstem response (ABR) thresholds (ABRthrs). The impaired ear median ABRthr was 55 dB nHL, where 40% had no recordable ABRthr. None of the subjects tested positive for congenital cytomegalovirus (CMV) infection. Fourteen subjects agreed to participate in magnetic resonance imaging (MRI). Malformations were common for all degrees of uSNHL and found in 64% of all scans. Half of the MRIs demonstrated cochlear nerve aplasia or severe hypoplasia and 29% showed inner ear malformations. Impaired ear and normal-hearing ear ABR input/output functions on a group level for subjects with ABRthrs < 90 dB nHL were parallel shifted. A significant difference in interaural acoustic reflex thresholds (ARTs) existed. In congenital uSNHL, MRI is powerful in finding a possible hearing loss cause, while congenital CMV infection may be relatively uncommon. ABRs and ARTs indicated an absence of loudness recruitment, with implications for further research on hearing devices. |
| first_indexed | 2024-03-09T03:19:07Z |
| format | Article |
| id | doaj.art-a5caf6e39d0c462995500177fed99440 |
| institution | Directory Open Access Journal |
| issn | 2077-0383 |
| language | English |
| last_indexed | 2024-03-09T03:19:07Z |
| publishDate | 2022-07-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Journal of Clinical Medicine |
| spelling | doaj.art-a5caf6e39d0c462995500177fed994402023-12-03T15:12:30ZengMDPI AGJournal of Clinical Medicine2077-03832022-07-011114396610.3390/jcm11143966A Prospective Study of Etiology and Auditory Profiles in Infants with Congenital Unilateral Sensorineural Hearing LossMarlin Johansson0Eva Karltorp1Kaijsa Edholm2Maria Drott3Erik Berninger4Division of Ear, Nose and Throat Diseases, Department of Clinical Science, Intervention and Technology, Karolinska Institutet, 141 52 Stockholm, SwedenDivision of Ear, Nose and Throat Diseases, Department of Clinical Science, Intervention and Technology, Karolinska Institutet, 141 52 Stockholm, SwedenDivision of Ear, Nose and Throat Diseases, Department of Clinical Science, Intervention and Technology, Karolinska Institutet, 141 52 Stockholm, SwedenDepartment of Audiology and Neurotology, Karolinska University Hospital, 141 86 Stockholm, SwedenDivision of Ear, Nose and Throat Diseases, Department of Clinical Science, Intervention and Technology, Karolinska Institutet, 141 52 Stockholm, SwedenCongenital unilateral sensorineural hearing loss (uSNHL) is associated with speech-language delays and academic difficulties. Yet, controversy exists in the choice of diagnosis and intervention methods. A cross-sectional prospective design was used to study hearing loss cause in twenty infants with congenital uSNHL consecutively recruited from a universal neonatal hearing-screening program. All normal-hearing ears showed ≤20 dB nHL auditory brainstem response (ABR) thresholds (ABRthrs). The impaired ear median ABRthr was 55 dB nHL, where 40% had no recordable ABRthr. None of the subjects tested positive for congenital cytomegalovirus (CMV) infection. Fourteen subjects agreed to participate in magnetic resonance imaging (MRI). Malformations were common for all degrees of uSNHL and found in 64% of all scans. Half of the MRIs demonstrated cochlear nerve aplasia or severe hypoplasia and 29% showed inner ear malformations. Impaired ear and normal-hearing ear ABR input/output functions on a group level for subjects with ABRthrs < 90 dB nHL were parallel shifted. A significant difference in interaural acoustic reflex thresholds (ARTs) existed. In congenital uSNHL, MRI is powerful in finding a possible hearing loss cause, while congenital CMV infection may be relatively uncommon. ABRs and ARTs indicated an absence of loudness recruitment, with implications for further research on hearing devices.https://www.mdpi.com/2077-0383/11/14/3966etiologysensorineural hearing lossunilateral hearing lossimagingcytomegaloviruselectrophysiology |
| spellingShingle | Marlin Johansson Eva Karltorp Kaijsa Edholm Maria Drott Erik Berninger A Prospective Study of Etiology and Auditory Profiles in Infants with Congenital Unilateral Sensorineural Hearing Loss Journal of Clinical Medicine etiology sensorineural hearing loss unilateral hearing loss imaging cytomegalovirus electrophysiology |
| title | A Prospective Study of Etiology and Auditory Profiles in Infants with Congenital Unilateral Sensorineural Hearing Loss |
| title_full | A Prospective Study of Etiology and Auditory Profiles in Infants with Congenital Unilateral Sensorineural Hearing Loss |
| title_fullStr | A Prospective Study of Etiology and Auditory Profiles in Infants with Congenital Unilateral Sensorineural Hearing Loss |
| title_full_unstemmed | A Prospective Study of Etiology and Auditory Profiles in Infants with Congenital Unilateral Sensorineural Hearing Loss |
| title_short | A Prospective Study of Etiology and Auditory Profiles in Infants with Congenital Unilateral Sensorineural Hearing Loss |
| title_sort | prospective study of etiology and auditory profiles in infants with congenital unilateral sensorineural hearing loss |
| topic | etiology sensorineural hearing loss unilateral hearing loss imaging cytomegalovirus electrophysiology |
| url | https://www.mdpi.com/2077-0383/11/14/3966 |
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