Page 92 - Portuguese Journal - SPORL - Vol 61. Nº1

Table 1
Indicators of risk of congenital permanent or late/progressive onset hearing loss in childhood

1 Family history of hearing loss in childhood and/or use of hearing aid before the age of 50 years
Family
2 Parental consanguinity
Intrauterine infections (CMV, herpes, rubella, syphilis, toxoplasmosis, chickenpox, and laboratory
3 Pregnancy findings of Zika infection in the mother and child)
Intrauterine exposure to toxic substances (cocaine and/or opioids) and tobacco
Neonatal suffering (Apgar 0-4 at 1 min or 0-6 at 5 mins, Ø spontaneous breathing at 10 mins,
4
hypotonia > 2 h after birth)
Perinatal NICU: hospitalization for > 5 days or ECMO or invasive ventilation or hyperbilirubinemia requiring
5
exchange transfusion
6 Weight at birth < 1,500 kg
Craniofacial anomalies. Especially anomalies involving the external ear (e.g., external ear atresia),
7
EAC, or temporal bone
Physical changes associated with syndromes that usually include conductive/sensorineural
8
deafness, such as the congenital white forelock (Waardenburg syndrome)
Congenital Neurodegenerative diseases (Hunter syndrome, leukodystrophies) or sensory-motor neuropathies
9
(Friedreich ataxia, Charcot-Marie-Tooth syndrome)
Syndromes associated with hearing loss (trisomy 21, 22q11 deletion, fetal alcohol syndrome,
10 neurofibromatosis, osteopetrosis, Usher syndrome, Waardenburg syndrome, Alport syndrome,
Pendred syndrome, Jervell and Lange-Nielsen syndrome)
Culture-confirmed infections associated with sensorineural hearing loss, including bacterial
11
and viral meningitis (especially herpes and chickenpox) and severe neonatal sepsis
12 Ototoxic substance administration > 5 days (gentamicin, trobramycin, or loop diuretics)
13 Recurrent or persistent (more than 3 months) otitis media
14 Chemotherapy (especially platinum derivatives)
Other
Caregiver’s concern about hearing loss or delayed speech/discourse development or learning
15
difficulties
Cranial trauma requiring hospitalization, especially involving the skull base or temporal bone
16
fracture
17 Ventriculoperitoneal shunt
Abbreviations: CMV, Cytomegalovirus; NICU, Neonatal Intensive Care Unit; APGAR, Appearance, Pulse, Grimace, Activity, Respiration;
ECMO, Extracorporeal membrane oxygenation; EAC, External Auditory Canal.
* Suggested alteration to the current recommendations.

h) Cranial trauma requiring hospitalization (up to school age) who had undergone
The AAP suggests that all children who ECMO or were exposed to intrauterine CMV
experienced cranial trauma requiring infection. Children diagnosed with bacterial/
hospitalization should have their hearing viral meningitis and severe neonatal sepsis
evaluated, in particular, but not exclusively, in who exhibited some degree of hearing loss
cases of temporal bone fractures, unlike what in the audiological evaluation at the time
is currently recommended in Portugal. 2–4 of discharge should also remain under
follow-up until school age, because of the
2. Timings of the audiological re-evaluation risk of worsening. The JCIH recommends
6
with otorhinolaryngology (ORL) consultation audiological re-evaluation up to nine months
(Table 2) of age in cases of other intrauterine infections
Based on the JCIH, we introduced the (other than CMV and Zika), hospitalization
recommendation of evaluating children in the NICU for longer than five days,

92 Portuguese Journal of Otorhinolaryngology - Head and Neck Surgery