Implementation of Newborn Hearing Screening in Albania.

Newborn hearing screening (NHS) was implemented in Albania in four maternity hospitals in 2018 and 2019. Implementation outcome, screening outcome, and screening quality measures were evaluated. Infants were first screened by midwives and nurses before discharge from the maternity hospital and returned for follow-up screening. Acceptability, appropriateness, feasibility, adoption, fidelity, coverage, attendance, and stepwise and final-referral rates were assessed by onsite observations, interviews, questionnaires, and a screening database. A post hoc analysis was performed to identify reasons for loss to follow up (LTFU) in a multivariate logistic regression. In total, 22,818 infants were born, of which 96.6% were screened. For the second screening step, 33.6% of infants were LTFU, 40.4% for the third, and 35.8% for diagnostic assessment. Twenty-two (0.1%) were diagnosed with hearing loss of ≥40 dB, six unilateral. NHS was appropriate and feasible: most infants are born in maternity hospitals, hence nurses and midwives could perform screening, and screening rooms and logistic support were supplied. Adoption among screeners was good. Referral rates decreased steadily, reflecting increasing skill. Occasionally, screening was repeated during a screening step, contrary to the protocol. NHS in Albania was implemented successfully, though LTFU was high. It is important to have effective data tracking and supervision throughout the screening.

NAL-NL2 Prescriptive Targets for Bone Conduction Devices With an Adaptation to Device Constraints in the Low Frequencies.

OBJECTIVES: To study the effectivity of a transformed NAL non-linear version 2 (NAL-NL2) gain prescription for percutaneous bone conduction devices (BCDs) and to investigate how to take into account output constraints for the lower frequencies. DESIGN: The NAL-NL2 prescription was converted to a bone conduction prescription rule. Adaptations were needed, as this converted rule prescribes more output at low frequencies than the device delivers. Three adaptations with different audibility and compression were compared. Setting 1 (S1, "optimal audibility") had most audibility due to adapted frequency-dependent compression, setting 2 (S2, "moderate audibility") had moderate output reduction below 1 kHz, and setting 3 (S3, "reduced audibility, least distortion") had most output reduction. Eighteen experienced BCD users rated their relative sound quality in paired comparisons for different sounds (own voice, mixed voices, traffic noise, and music). In addition speech intelligibility in quiet and noise were assessed. RESULTS: The relative sound quality rating for the adapted prescriptions varied between the stimuli: more low-frequency sound was preferred for music (S1 over S3), and less low-frequency sound was preferred for the own voice (S2 and S3 over S1). No differences in quality rating were found for mixed voices or traffic noise. Speech intelligibility in quiet scores at 45 dB SPL was significantly lower for S3 than for S1. Speech intelligibility in noise was significantly reduced in all settings and S3 yielded significantly better speech intelligibility in noise than S1. CONCLUSIONS: With a moderate gain reduction for low frequencies to comply with device constraints the transformed NAL-NL2 prescription was found suitable for fitting BCDs. Perceived sound quality depended on the gain settings, but also on the sound spectra and how the sound was appreciated. A moderate gain reduction below 1 kHz seems to be the optimal adaptation as it has a neutral or positive relative sound quality for all stimuli without negative effects on Speech intelligibility. The NAL-NL2-BC prescribed a sufficient amount of gain, as indicated by the speech tests.

Implementation of a neonatal hearing screening programme in three provinces in Albania.

OBJECTIVES: The EUSCREEN study compares the cost-effectiveness of paediatric hearing screening programmes and aims to develop a cost-effectiveness model for this purpose. Alongside and informed by the development of the model, neonatal hearing screening (NHS) is implemented in Albania. We report on the first year. METHODS: An implementation plan was made addressing objectives, target population, screening protocol, screener training, screening devices, care pathways and follow up. NHS started January 1st, 2018 in four maternity hospitals: two in Tirana, one in Pogradec and one in Kukës, representing both urban and rural areas. OAE-OAE-aABR was used to screen well infants in maternity hospitals, whereas aABR-aABR was used in neonatal intensive care units and in mountainous Kukës for all infants. Screeners' uptake and attitudes towards screening and quality of screening were assessed by distributing questionnaires and visiting the maternity hospitals. The result of screening, diagnostics, follow up and entry into early intervention were registered in a database and monitored. RESULTS: Screeners were keen to improve their skills in screening and considered NHS valuable for Albanian health care. The number of "fail" outcomes after the first screen was high initially but decreased to less than 10% after eight months. In 2018, 11,507 infants were born in the four participating maternity hospitals, 10,925 (94.9%) of whom were screened in the first step. For 486 infants the result of screening was not registered. For the first screen, ten parents declined, eight infants died and one infant was discharged before screening could be performed. In 1115 (10.2%) infants the test either could not be performed or the threshold was not reached; 361 (32,4%) of these did not attend the second screen. For the third screen 31 (34.4%) out of 90 did not attend. Reasons given were: parents declined (124), lived too far from screening location (95), their infant died (11), had other health issues (7), or was screened in private clinic (17), no reason given (138). CONCLUSIONS: Implementation of NHS in Albania is feasible despite continuing challenges. Acceptance was high for the first screen. However, 32.4% of 1115 infants did not attend the second screen, after a "fail" outcome for the first test.

Auditory brainstem response morphology and analysis in very preterm neonatal intensive care unit infants.

OBJECTIVES/HYPOTHESIS: Analysis of auditory brainstem response (ABR) in very preterm infants can be difficult owing to the poor detectability of the various components of the ABR. We evaluated the ABR morphology and tried to extend the current assessment system. STUDY DESIGN: Prospective cohort study. METHODS: We included 28 preterm very low birth weight infants admitted to the neonatal intensive care unit of Sophia Children's Hospital. ABRs were measured between 26 and 34 weeks postconceptional age. The presence of the following ABR parameters was recorded: the ipsilateral peaks I, III and V, the contralateral peaks III and V, and the response threshold. RESULTS: In 82% of our population, a typical "bow tie" response pattern was present as a sign of early auditory development. This bow tie pattern is the narrowest part of the response wave and is predominantly characterized by the ipsilateral negative peak III. This effect may be emphasized by the contralateral peak III. The bow tie pattern is seen approximately 0.1 milliseconds before the ipsilateral peak III. From 30 weeks postconceptional age onward, a more extensive morphologic pattern is recorded in 90% of the infants. A flow chart was designed to analyze the ABR morphology of preterm infants in an unambiguous stepwise fashion. CONCLUSIONS: A typical bow tie pattern preceding peak III seems to be the earliest characteristic of the developing ABR morphology in preterm infants. As ABR characteristics will improve with increasing age, neonatal hearing screening should be postponed until after 34 weeks.

Audiological profile of children and young adults with syndromic and complex craniosynostosis.

OBJECTIVES: To determine syndrome-specific type, severity, and prevalence of hearing loss to facilitate follow-up and treatment. DESIGN: Tertiary pediatric hospital craniofacial clinic survey study. If insufficient or no data were available for a child, he or she was referred to an audiologist for pure-tone audiometry. SETTING: Academic research facility. PATIENTS: Information was gathered regarding 132 children and young adults with craniosynostosis. MAIN OUTCOME MEASURES: The primary outcome was hearing assessment of children and young adults with various types of craniosynostosis. A secondary outcome was inference regarding the incidence of otitis media among children and young adults with craniosynostosis. RESULTS: We found mild or moderate hearing loss in 44.0% of patients with Apert syndrome, in 28.5% with Crouzon syndrome, in 62.1% with Muenke syndrome, in 28.6% with Saethre-Chotzen syndrome, and in 6.7% with complex craniosynostosis. Hearing loss was conductive in most patients with Apert, Crouzon, and Saethre-Chotzen syndromes and it was predominantly sensorineural in patients with Muenke syndrome. Sensorineural hearing loss at lower frequencies was found only in patients with Muenke syndrome. CONCLUSIONS: Most patients with syndromic and complex craniosynostosis have recurrent otitis media with effusion, causing episodes of conductive hearing loss throughout their lives. Sensorineural hearing loss can occur in all 4 syndromes studied but is the primary cause of hearing loss in children and young adults with Muenke syndrome. For patients with these syndromes, we recommend routine visits to the general practitioner or otolaryngologist, depending on national standards of care, to screen for otitis media with effusion throughout life. We also advise early screening for sensorineural hearing loss among children and young adults with these syndromes.