Title of the case study: IBS

1. General data on the case:

– Biographical and case identification data –

IBS is the first child of a young couple, currently living near Chaves (interior north of Portugal). She has an 8-month-old baby brother. She lives at her maternal grandparents’ home.

– Case history (Anamnesis):

  • Significant medical data

IBS was born on 29-7-2016, in a central hospital in the city of Porto, with a history of prematurity (36 weeks) and low birth weight (2300g). She has been monitored in Neonatology since the 1st month of life, due to intrauterine growth restriction of placental cause.

She did OAE’s when she was discharged from the maternity hospital, giving a REFER result in this Universal Neonatal Hearing Screening. She repeated again the OEA’s and had an ABR showing electrophysiological thresholds of 70dB in the right ear and 80dB in the left ear, with type A tympanogram (on 12/21/2016, at 5 months of age). She repeated ABR’s at 8 months of age, with no detectable waves at 100dB in the right ear and wave V thresholds at 80dB in the left ear, being proposed for candidacy for Cochlear Implants and consultation of Genetics and Pediatrics. There is no family history of hearing loss in the family. She started using hearing aids at 9 months of age. During this period she did not benefit from any support in speech therapy. She waited for the public hospital’s decision to proceed to cochlear implants until January 2018, having opted to consult a second opinion at our Lusíadas Porto Hospital’s Deafness, Vertigo and Tinnitus Center.

In March 2018 (at the age of 19 months), she started support in Auditory-Verbal Therapy with a certified therapist. In the space of 2 months we had diagnostic information that did not have any functional gain for access to speech sounds with the hearing aids, being confirmed with conditioned audiometric tests. In June 2018 she undergoes simultaneous cochlear implant surgery and on July 19, at 23 months of age, she activates both implants.

Currently, she is 4 years and 9 months of chronological age and 2 years and 9 months of using cochlear implants.

  • Significant psychological data

IBS has cognitive performance as expected for the age, communication is mediated only by audition and spoken language, without needing support in gestures or support in sign language. From a behavioral point of view, IBS presents itself as a girl with a difficult relationship, with a very sensitive temperament (low frustration tolerance) and with a communicative profile of own agenda type.

  • Educational setting

At 6 months of age, she entered the day care center, in a nursery, with hearing peers. Currently she stays at the same school, with children of her age group, supported by the Local Early Intervention Team and a speech therapist in the school context, once a week, in addition to the Auditory-Verbal Therapy sessions.

  • Significant social data

The family is of the lower-middle class, both parents are employed. They do not currently live in their own home but share the home of their maternal grandparents. Spoken Portuguese is the only means of expression used in the family context.

  • Age of fitting the first hearing aids and cochlear implants

Hearing aids at 9 months of age and Cochlear Implants (Cochlear Kanso) at 23 months of age.

  • How often and what kind of support/rehabilitation does the child/family receive?

Until 19 months of age, IBS only had pediatric development consultations at the public hospital and had no specific support in speech therapy. He started sessions based on the Auditory-Verbal Therapy approach at 19 months of age, having started with complementary local support (in the current region where they live), with speech therapy in the school context until March of 2020. Since then, she only benefits from tele practice sessions with a LSLS Cert. AVT, one session per week.

3. The implantation

  • age of undergoing first cochlear implantation (left or right side): 23 months
  • type of implant: Cochlear Kanso 1
  • number of implants (CI and hearing aids): 2 HA’s from 9 to 23 months and 2 CI’s since then

current average duration of the CI use (information from the audiologists): currently, 10 hours on both sides (datalogging information: January 2021)

  • other relevant information: difficult periods during early-stage development. IBS always struggled to have her hearing aids and cochlear implants on. After her cochlear implantation, and according to her parents, she changed a lot her personality, by having lots of tantrums and a difficult acceptance of adults orientations. Her CI’s programming’s were very difficult during the first 9 months and only after this period she started using her CI’s in a systematic and all waking hours. Nowadays she has a stable aided audiogram, with 35dB average thresholds on both sides.

4. What is the procedure to start the speech therapy after CI?

Parents/caregivers are present and active participants in Speech Therapy sessions after CI’s implantation and activation.

5. The Speech Therapy used in the rehabilitation

After being duly informed about the various communicative modalities and expected results according to the literature, the family continued to intervene in our Center because it is the only one in Portugal with a certified therapist in Auditory-Verbal Therapy in the country. The preferred means of communication was the language spoken through hearing.

  • frequency and duration of therapy

She benefits from 1 session per week; the duration of each session is around 1 hour-1 hour and 30 minutes maximum, in one-to-one therapy and in presence mode; after march 2020, sessions began with telepractice until now (1 hour per session), and every 6 months we have one session in presence for evaluation.

  • listening development

Currently, she can locate sounds in all relative positions in space; clearly identifies all 6 Ling sounds, by repeating them; features a 3-item auditory memory; she is able to attend 2 non-related commands and 3 sequential commands in a routine task.

  • speech production development

IBS speech production development is her main issue currently, after almost 2 years and 9 months of CI’s activation, she struggle to have an intelligible speech production… After almost 18 months of activation, IBS wasn’t performing very well and she was diagnosed with Childhood Apraxia of Speech (CAS). Currently, she is capable of producing words with 2 syllables and, occasionally, with 3 syllables; Most of the times she is able to produce words with the following phonemes: /p, b, m, t, d, n, R, sh/, while /f, v, s, K/ are emergent sounds in her spontaneous speech. She has lots of phonological processes, with phonemic substitutions, omissions and syllabic reductions.

  • spoken language development.

Currently, she is able to speak at least with 3 words in a phrase.

  • spoken language supported by signs or other visual cues: She does not use any support in gestures, only use spoken language as a means of communication.
  • monitoring of the progress in auditory, speech and spoken language development. Currently, IBS is followed in audiology every 6 months, and the last schedule review was carried out in January 2021, with mean thresholds of 35dB in both ears. It makes periodic assessments of your language development every 6 months, in person.

6. Results

Strengths Hearing loss was diagnosis early, before 6 months of age, by the gold standards of JCIH (Joint Committee of Infant Hearing)No additional needs      Weaknesses What are the case study de facto weaknesses?What can be improved or done different, given the current situation. Challenges  
Opportunities The support programmes used.The engagement of the family. What trends or conditions may positively influence the case?  Threats Unexpected resultsWhat to avoid? Poor services What trends or conditions may negatively influence the case?        

Strengths

  • What are the case study de facto strengths?
    • Hearing loss was diagnosis early, before 6 months of age, by the gold standards of JCIH (Joint Committee of Infant Hearing)
    • No additional needs
    •  
  • Which were the strengths of the rehab?
    • Parents are truly involved in therapy sessions and improving their strategies and techniques during the journey so far
  • The collaboration among the actors involved.
  • Factors of success:
    • IBS had access to the most up-to-date hearing technology (first hearing aids and second cochlear implants)
    • Parents were able to choose the brand and model of CI’s.
    • Parents are systematic participants in all therapy sessions.

Weaknesses

  • What are the case study de facto weaknesses?
    • IBS, although with a fairly early diagnosis of permanent hearing loss, didn’t had access to a proper habitation process, conducted by a specialized team. She only had access to hearing technology and no indications on how to evaluate her auditory access and language development.
    • She only had her CI’s almost with 2 years of age, even with a early diagnosis of permanent hearing loss.
    • Parents limited time to re-schedule therapy sessions (in case of a lost sessions) and to be fully available for her during a session (often, the mother has the little sibling in her lap while therapy is happening, in telepractice sessions).
    • During this period of CI using, she already had several speech processor failures.
    • She had complementary sessions of speech therapy in presence, to work speech production (because of CAS diagnostic) in presence until March of 2020 but with the pandemic situation, she stopped with this specific support due to restrictions of access of the professional in the school context.
  • What can be improved or done different, given the current situation.
    • We discussed several options to move the day and timetable for therapy sessions, but both parents have limited time to schedule other day for therapy during the week.
  • Challenges
    • She has a special difficulty in terms of her speech production, which makes her to struggle to have good opportunities for a sustained conversation with adults and with her peers.
    • Her behaviour management in telepractice sessions is challenging, amplified after his little brother was born.