Management of children with auditory neuropathy spectrum disorder (ANSD)

Gökdoğan, Çağıl; Altınyay, Şenay; Gündüz, Bülent; Kemaloğlu, Yusuf Kemal; Bayazıt, Yıldırım A.; Uygur, Kemal

doi:10.1016/j.bjorl.2015.08.022

Cited by 4 publications

(7 citation statements)

References 26 publications

(53 reference statements)

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“…Our prevalence of patients with hyperbilirubinemia was 28%, near the middle of the range reported in the literature (15% in Gökdoğan et al 2016, 20% in Roche et al 2010, 29.8% in Ching et al 2013) but lower than in others (44.3% in Berg et al 2005, and 48.4% in Berlin et al 1998).…”

Section: Discussionsupporting

confidence: 46%

A 15-Year Review of 260 Children With Auditory Neuropathy Spectrum Disorder: I. Demographic and Diagnostic Characteristics

Morlet

Parkes²,

Pritchett³

et al. 2023

Ear &Amp; Hearing

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Objectives: The aim of the study was to review the demographic and clinical characteristics of all pediatric patients diagnosed with auditory neuropathy spectrum disorder (ANSD) by a pediatric health care system from 2005 to 2020 and examine whether or not our diagnostic capabilities in an ANSD population have evolved as our institutional experience has grown and knowledge in the field has expanded. Design: This was a retrospective study reviewing the demographic data, medical history, imaging studies, audiological and speech-language data, type of audiological intervention and mode of communication in 260 pediatric patients diagnosed with ANSD over a 15-year period. Results: The study revealed that male and female children were equally affected with all levels of hearing detection being represented and that about 40% of affected children were premature and most were admitted to the neonatal intensive care unit. More than a third of our patients presented with a complex medical history and/or neural involvement while about 30% were full-term newborns with normal pregnancy, no prenatal complications or infections, normal birth weight, no neonatal intensive care unit need, no hyperbilirubinemia, no respiratory distress requiring ventilation, and no known syndrome. Review of audiological findings confirms that otoacoustic emissions are not always present in ANSD cases, and that the presence of an abnormal wave V on the auditory brainstem response tracings (only present at high intensities and with an absent intensity/latency function) is not a rare finding and should not immediately be dismissed as not being a case of ANSD. Conclusions: This review of ANSD diagnosis over a 15-year period clearly reveals the drastic improvements made in the identification of ANSD, with a drastic decrease in the age at diagnosis and a reduction in the percentage of misdiagnosed patients. The study also stresses the need for continued improvement in different areas such as genetic studies and physiological measures to help clinicians distinguish between pre- and postsynaptic ANSD.

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Section: Discussionsupporting

confidence: 46%

A 15-Year Review of 260 Children With Auditory Neuropathy Spectrum Disorder: I. Demographic and Diagnostic Characteristics

Morlet

Parkes²,

Pritchett³

et al. 2023

Ear &Amp; Hearing

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“…30,31 Auditory neuropathy is a rare condition reported in only 8% of children per year who are newly diagnosed with hearing loss. 32 Otoacoustic emissions testing used in most American states as part of their universal hearing screening program will not identify auditory neuropathy. The recognition of onset of auditory neuropathy without performing an auditory brainstem response may be missed in infants and young children, especially those with extensive comorbidities.…”

Section: Discussionmentioning

confidence: 99%

The Clinical Journey of Patients with Riboflavin Transporter Deficiency Type 2

Amir

Atzinger

Massey³

et al. 2019

J Child Neurol

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Purpose: To identify symptoms and health care interactions with patients with riboflavin transporter deficiency (RTD) type 2 prior to diagnosis. Methods: Parents of children with riboflavin transporter deficiency type 2 (n = 10) were interviewed to collect data on the patient’s clinical journey. Results: The average diagnostic delay was 27.6 months. Neurologists were the most commonly visited clinician (90%). Common symptoms during the first year of the patient’s clinical journey included abnormal gait and/or ataxia (70%), nystagmus (50%), and upper body muscle weakness (40%). Prior to diagnosis, optic atrophy, sleep apnea, breath-holding spells, and dysphagia were commonly observed. Hearing loss was only reported in 40% of subjects prior to diagnosis. Riboflavin responsive megaloblastic anemia is reported for the first time. Mitochondrial disease was the most common suspected diagnosis (30%). Conclusion: Despite clinical variability, common early symptoms of riboflavin transporter deficiency type 2 exist that can better allow clinicians to more rapidly identify riboflavin transporter deficiency type 2.

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“…93 In addition, the ABR is normal, while the OAEs are normal in ANSD. 69 Maternal smoking or drug use may result in false positive OAE testing while passing ABR testing, which may be associated with conductive hearing loss. 94 With CMV infection-related HL, the sample collection time is time-sensitive to allow timely antiviral treatment.…”

Section: Reasons For Hearing Screening Failurementioning

confidence: 99%

“…AABR has high specificity and can reduce the referral rate, thereby reducing the cost of further diagnosis. A study has proposed changing the two-step OAE to AABR alone, which is worthy of our further thought and evaluation 69 ( Table 1 ).…”

Section: Neonatal Hearing Loss–related Screeningmentioning

confidence: 99%

Change to Hearing Loss–Related Risks and Screening in Preterm Infants

2020

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Hearing loss is one of the most common congenital defects in infancy; it increases speech and language delays and adversely affects academic achievement and socialemotional development. The risk of hearing loss in premature infants is higher than that in normal newborns, and because of the fragility of the auditory nervous system, it is more vulnerable to different risk factors. The hearing screening guidelines in current use were proposed by the American Academy of Pediatrics and updated in 2007, but there are no uniform guidelines for hearing screening in preterm infants. This review focuses on the risk factors related to hearing loss in premature infants, hearing screening strategies, and reasons for failure. The aim is to provide a more comprehensive understanding of hearing development in preterm infants to achieve early detection and early intervention. At the same time, attention should be paid to delayed auditory maturation in preterm infants to avoid excessive intervention. Key Points

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Management of children with auditory neuropathy spectrum disorder (ANSD)

Cited by 4 publications

References 26 publications

A 15-Year Review of 260 Children With Auditory Neuropathy Spectrum Disorder: I. Demographic and Diagnostic Characteristics

A 15-Year Review of 260 Children With Auditory Neuropathy Spectrum Disorder: I. Demographic and Diagnostic Characteristics

The Clinical Journey of Patients with Riboflavin Transporter Deficiency Type 2

Change to Hearing Loss–Related Risks and Screening in Preterm Infants

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