Title of the case study: Bilateral sequential cochlear implantation in an amaurotic child

1. General data on the case:

Biographical and case identification data

  • Gender: Male
  • Date of birth: 11.05.2003
  • Current age: 17 years-old
  • Address: Rua Ramiro Martins N.11-R/C-Esq-Poente, 4480-Arcos, Vila do Conde, Portugal
  • Family composition: Nuclear family consisting of a father, mother, and their children (two twin boys).

Family History:

  • Family hearing status: no history of congenital or acquired hearing loss;
  • No consanguinity;
  • No known genetic disorders;
  • Father: healthy; superior education; architect;
  • Mother: depression; superior education; 2nd grade teacher.

Case history (Anamnesis):

Significant medical data


  • G:1/P:1;
  • Planned and accompanied twin pregnancy;
  • Close follow-up;
  • Negative serology tests during 1st, 2nd, and 3rd trimesters;
  • Normal echography during 1st, 2nd, and 3rd trimesters;
  • Pre-eclampsia diagnosed in the 3rd trimester (week 29).


  • Mother´s age at delivery: 25 years old;
  • Premature eutectic delivery at 29 weeks into the pregnancy; very low birth weight (915g); APGAR (2/0); neonatal resuscitation;
  • Neonatal Intensive Care Unit: early onset of neonatal sepsis with the administration of intravenous antibiotics;

Neonatal Hearing Screening: not implemented at the time in our country. At 2 months of age auditory brainstem response (ABR) revealed profound bilateral deafness.

Development in the first year of life:

  • First smile: 1 month; Head control: 6 months; First steps: 20 months (with the physiotherapist’s assistance); Surgical interventions:
  • Ophthalmological surgery in the United States of America at 2 years of  age;
  • Cochlear implantation surgery at 3 and 4 years of age.

National vaccination program: Updated.

Childhood illnesses: None.

Current state of health: Bilateral amaurosis – complete retinal detachment.

Significant psychological data

Cognitive development: Mild cognitive impairment.

  • School degree: currently in high school in a reference school for blind students.
  • School performance: average.
  • Additional needs: writes and reads in Braille.
  • Communication in spoken language: oral communication.
  • Social skills: maintains and grows relationships with colleagues and the community.

Educational setting: special education, unit in mainstreamed school, full mainstreaming.

  • Special education: reference school for blind students.

Significant social data

Socio-economic and cultural conditions of the family: moderate-income family; supportive, affective, and encouraging household;

Age of fitting the first hearing aids and cochlear implants

  • Age of fitting hearing aids: 2 years of age
  • Age of cochlear implants: 3 years of age – right side. 4 years of age – left side.
How often and what kind of support/rehabilitation does the child/family receive?

The child was first seen at the reference cochlear center when 2 and a half years old. Profound bilateral neurosensorial hearing loss was confirmed. Before cochlear implantation, the family was introduced to the rehabilitation process through a formal evaluation done by the speech and language therapist and audiologist who explained the process ahead. The first cochlear implant surgery was done on the right side, at 3 years old with biweekly otolaryngologist consultations during the first month. Speech and language rehabilitation occurred four times a week within the first three months, after which he continued with 2 to 3 sessions a week with a speech and language therapist in the area. At 4 years of age, sequential left cochlear implant surgery was performed.

The implantation

  • age of undergoing first cochlear implantation (left or right side): Sequential cochlear implantation – right ear at the age of 3 and then the left ear at 4;
  • type of implant: CI24RE(CA)
  • number of implants (CI and hearing aids): 2 implants
  • current average duration of the CI use (information from the audiologists): 6.8 hours
  • other relevant information: difficult periods during early-stage development. If yes, why?

What is the procedure for starting speech therapy after CI?

After the activation of the first cochlear implant, an intensive process of auditory habilitation began, in which the child had speech therapy four times a week, lasting approximately two and a half hours. This process took place at the implantation center. During this period, adjustments were made to the speech processor programming by the audiologist. After this phase, the child returned to the area of residence, having speech therapy two to three times a week.

The Speech Therapy used in rehabilitation

  • describe the kind of therapy done (communication options, approach, environments, average timing): after cochlear implantation, the family opted for oral verbal communication, having decided on a direct approach with the child. Speech therapy took place in a school environment, either in the classroom or individually.
    • frequency and duration of therapy: the therapy took place every two weeks, lasting approximately 45 minutes.
    • listening development: due to significant psychomotor agitation, reduced attention span, and personality traits, the initial auditory development was slow. After the child began to distinguish words and phrases, approximately one year after implantation, he began to show a great interest in hearing, and if he was disconnected or removed from one of the cochlear implants that would induce a state of agitation, to the point of even hurting himself. Subsequently, around the age of 10, he purportedly resorted to self-harm when left without the implants, which reveals a great sense of frustration in the absence of sound.
    • speech production development: at the age of 11, he was able to produce the sounds /p, b, m, t, d, j, ch/
    • spoken language development: From 3 to 5 years of age, the boy acquired a reduced receptive and expressive vocabulary. He was able to produce onomatopoeia and isolated words. Around the age of 5, despite presenting some expressive vocabulary and understanding its communicative functionality, his speech was characterized by echolalia and, more rarely, isolated words. His vocabulary continued to grow and at 9 years old he built sentences with 2 content words. His speech was unintelligible to strangers. At the age of 10, he continued to show an increase in expressive vocabulary and echolalia. At the age of 11, his sentence construction already contained 3 content words as well as echolalia.
    • spoken language supported by signs or other visual cues? Use of sign language? (if any): at the age of 6, “alphabetic writing on the palm of the hand” was introduced as a means of facilitating the acquisition of speech sounds. At the age of 14, he started learning Braille.
    • monitoring of the progress in auditory, speech, and spoken language development. How?

After the initial auditory qualification period, the child was accompanied every three months to monitor the development of his auditory, linguistic and communicative abilities, with an assessment performed by the audiologist and a formal and informal assessment by the speech therapist, in order to better understand the communicative profile of the child. In addition to the formal tests, checklists submitted by the parents were also used, together with reports from them as well as the teachers and speech therapists in the area of residence.

  • other relevant information


Child’s personalityIntensive auditory initial habilitation
Attention / ConcentrationPsychomotor agitationDietary changes
The support programs used.The engagement of the family.
Frequent breakdowns of speechprocessorsPoor servicesFamilial instability