We present the case of a 7-year-old boy who was diagnosed with plurimalformative syndrome at birth, presenting clinical features that could not be included in a specific syndrome. He was born at term after an uneventful pregnancy (mother followed isotretinoin therapy in the first two weeks of pregnancy), spontaneous delivery, birth weight = 3380 grams, APGAR score 10. A craniofacial dysmorphism was discovered: agenesis of the right auricle, severe dysplasia of the left auricle, bilateral external auditory canal atresia, microretrognathia, broad nose implantation - hypertelorism, mongoloid palpebral fissures, severe hypoplasia of the right and moderate hypoplasia of the left upper jaw, palpebral fissures asymmetry (left < right), lagophthalmia (2-3 mm bilaterally), nasolabial asymmetry (more significant on the right), oculomotricity deficiency (right eye abduction paralysis), clinodactyly of the fifth finger, bilateral flat foot.
Temporal bone computed tomography scan revealed dysplastic ossicles, unidentifiable stapes, and oval window, hypopneumatization of mastoid cells, hypoplasia of the tympanic cavity, without changes in the internal ear. The contour of the facial nerve in the mastoid segment was not clearly defined.
Following the investigations, the diagnosis of moderate conductive hearing loss in the left ear and severe mixed hearing loss in the right ear was established. On auditory brainstem response, the V wave was present at 70 dB HL in the right ear and 80 dB HL in the left ear. From 1 month and a half, hearing rehabilitation was made with bilateral BAHA Softband. Audiological reevaluation at age 4 revealed the following audiological profile: Auditory brainstem responses (ABR) on bone conduction - V-wave present at 10 and 20 dB HL in the left ear (LE) and at 20 and 30 dB HL in the right ear (RE), auditory steady-state response (ASSR) - between 60 and 70 dB HL in the LE and 80 and 90 dB HL in the RE.
SmartEP Intelligent Hearing System (IHS) equipment (Miami, Florida, USA) was used for ABR and ASSR. Air conduction was tested with headphones, for atretic ears, and with a B-71 transducer for bone conduction, held by one finger on the mastoid.
At 6 years and 8 months, a BAHA Attract was implanted on the left ear and three months later on the right ear. At 7 years and one month, we recorded the cortical auditory evoked potentials on the aided condition to quantify the maturation of the central auditory pathways.
The P1 CAEP records were obtained after 90 minutes of testing in a soundproofed room. The patient was placed on a chair, and he watched cartoons without sound during the procedure. The patient was using a bilateral BAHA Attract implantable prosthesis. The electrodes were placed according to the norms of the International Electrode System 10-20: the active Cz electrode was connected to the positive input of the amplifier, the reference electrode was positioned on the mastoid of the ear, and the ground electrode was placed at Fpz. To minimize the ocular artifacts, a supraorbital electrode was used, paired with an infraorbital reference electrode placed ipsilaterally. The level of impedance of the electrodes was maintained between 1-3 kOhms. A calibrated loudspeaker placed at 1 m distance in 0  angle emitted a speech stimulus, the "ba" syllable, at 70 dB nHL intensity. The stimulus rate was 1.10/s, duration 114875 microsec, for 512 sweeps, artifact rejection criterion at +- 100 microV. The stimulus CAEPSs recorded in response was analyzed by a SmartEP USB software from the Intelligent Hearing System.
The check-up audiogram performed at 7 years and 4 months of age showed hearing thresholds between 20 and 30 dB HL for the RE.
Neuropsychological functioning was performed at the age of 7 years and one month, by a clinical neuropsychologist using the Wechsler Intelligence Scale for Children - Fourth Edition (WISC-IV), adapted in Romania in 2012.
The assessment was based on oral/acoustic modality, which refers to the use of spoken language with auditory amplification (Baha Attract System), without visual instructions; the administration was possible without modifications, and all the tests were applied according to a standard procedure. The patient was administered ten subtests of the Wechsler Intelligence Scale for Children - Fourth Edition (WISC IV). WISC-IV measures general intelligence and specific indexes, including verbal comprehension, perceptual reasoning, working memory, and processing speed.
Verbal Comprehension Index (VCI) measures verbal concept formation. The subtests included are Similarities, Vocabulary and Comprehension.
Perceptual Reasoning Index (PRI) measures non-verbal and fluid reasoning. The subtests included are Block Design, Picture Concepts, and Matrix Reasoning.
Working Memory Index (WMI) measures working memory. The subtests included are Digit Span and Letter-Number Sequencing.
Processing Speed Index (PSI) measures the speed of information processing. The subtests included are Coding and Symbol Search.
The P1 wave was observed with normal morphology and a delayed latency (175 ms) for this age (Figure 1). No artifacts caused by BAHA were observed, as described by Rahne et al.. This latency value indicates that there is a delay in the maturation of the central auditory pathways.