1. INTRODUCTION

1.1 First contact with the patient

A one-year-old girl attends to the ENT specialist due to her parent´s suspicions of a hearing loss, even considering the normal results of the measure of evoked potentials. They need to know if their daughter has a hearing impairment. They have seen on TV how a device allows the deaf to hear and it is more effective the sooner it is implanted. They state their distress facing the possibility of having a hearing impaired daughter who is not benefiting from that device.

The ENT specialist proceeds to the examination of the girl´s pinna, external ear canal and the tympanic membrane, without finding any particular anomaly. Then, an observation of the patient behavior towards sound stimuli is carried out. The absence of response raises the suspicions of a hearing pathology. The specialist collects information about any possible deafness background in the family, the gestation period and delivery, the girl evolutionary development from birth to the current moment, the illnesses suffered and its medical treatment, etc. None of this data seems to be of some relevance. Therefore, the specialist informs the family that a full study of the girl will be undertaken and about the need to run hearing tests (audiometric assessment). Information about the cochlear implant is also provided to help the parents to manage the distress produced by the lack of knowledge about this device. The ENT specialist refers the family to the Speech Therapist specialized in deafness and member of the implant team.

1.2 First visit to the Speech Therapist from the implant center.

The Speech Therapist collects all the information provided by the ENT specialist interview with the family.

2. DATA COLLECTION FROM OBSERVATION OF THE PATIENT

2.1 Observation of the behavior.

– The girl has a friendly character.

– She keeps the attention and is easy to get the eye tracking with her.

– She is attracted by visual stimuli and quickly generates an empathetic relationship with the Speech Therapist.

2.2. Observation of the psychomotor development.

– The girl understands object permanence.

– She mimics the adults´ grimaces.

– She is able to throw and pick up small objects.

– The girl grabs the objects shown to her and is able to drop them, proving a sensorimotor coordination of fine movements.

– With the help of an object, she is able to stand up and seek help from her parents to walk.

2.3 Observation of communication skills and language.

– The communication skills of the girl are limited to some natural gestures to satisfy her basic needs such as hunger, sleep, thirst or hygiene that the parents are able to understand.

–   She lacks a system of communication that would allow her to acquire concepts.

–  She makes sounds without semantic content.

2.4 Observation of the hearing capability

– There is not response to musical instruments with different frequency characteristics like drums, bells, xylophone, tambourine, flute, etc, even in the higher intensity.

– There is neither a response to musical toys or to other noise.

– With regard to the human voice, she does not response to her name or reacts to screaming or strong vocalic sounds.

2.5. Observation of the family

– Both parents are coming to the visits. They look anxious but willing to cooperate.

– The communicative interaction with her daughter is limited to the basic needs, being ahead of them sometimes.

– The socioeconomic level of the family is medium-high, with both parents having steady jobs.

–  They ease the communication with the Therapist showing their interest and understanding the information provided.

– With regard to the family environment, they recognized their total involvement with the girl since they start suspecting the hearing loss.

– They have another nine month old child who interacts with his sister.

3. CONCLUSIONS OF INITIAL ASSESSMENT

This is a case of a potential candidate for a cochlear implant waiting for diagnostic tests. Therefore, the parents are provided with all the information related to deafness and to the cochlear implant and they are also reassured. A new appointment is made to discuss about the results of the next hearing-test.

4. SECOND INTERVIEW WITH THE SPEECH THERAPIST

The parents come back to the speech therapist with the results of the hearing tests made by an audiologist. The ENT specialist has already provided the speech therapist with details of these results. The audiometric assessment analyzed the reflex response of the patient and included a visual reinforcement tone audiometry. The results showed a diagnosis of bilateral hearing loss. The family is advised to conduct an appropriate prosthesis adaptation to assess if there is any residual hearing. They will visit the speech therapist again once the adaptation is finished and they will get a new appointment if the results are normal.

The speech therapist informs them about the correct operation and use of the hearing aids. They also receive information about how to encourage their daughter’s response towards the sounds and how to manage problems such as rejection, device whistling, etc.

Once the prosthetic adaptation is finished, there are not auditory responses or behavioral changes in the girl or any sign of the existence of any useable residual hearing.  The final decision is to make a cochlear implant and start with the pre-implant treatment.

5. START OF THE TREATMENT : PRE-IMPLANT

The girl goes with her parents to the treatment sessions once per week forty five minutes until the surgical intervention.

There is a need to initiate the girl and the family to sign language due to the characteristics of the girl, without any communicative system. This will be done using the bimodal bilingualism. Signs are used while playing and interacting with the parents. An educational video is provided with the aim of accelerate the proper learning of the sign language by parents and close members of the family.

The progress is fast as the girl keeps her attention and participates effectively in the sessions.

The professional speaks with the family at the end of the speech therapy session, not using the same space to do so. The therapist explains the objective of the sessions; the type of collaboration expected from the parents and tries to clarify any possible question about the intervention, the treatment and the possible reaction of the girl after the implant.

There is not any contact with other professionals since the girl has not been treated so far and does not go to any educational center.

6. THE SURGERY AND FIRST CONECCTION

The girl is admitted to the hospital and she will be operated by the ENT surgeon to insert the cochlear implant in the right ear. The intervention is successful; an optimal insertion of an electrode in the cochlea is achieved as shown in the post-surgical radiograph.

She remains hospitalized for four days and will take antibiotic for a week. After seven days, she visits the doctor to remove the staples that fixed the flap and it looks good.

After one month and twelve days waiting, the girl goes to the speech therapist where the technician makes the first connection in the presence of the parents, the therapist and an ENT specialist from the medical team. All the electrodes are connected using a Continuous Interleaved Sampling (CIS) and following certain stimulation parameters predefined, since the girl cannot offer any information about the acoustic stimulation received. The only response observed is the movement of eyes and head when the electrodes are stimulated. Once the programming is done, the MAP is recorded in her speech processor, and even though there is some rejection at first, it is successfully completed.

All the process is explained to the family, and once all the pieces of the cochlear implant are reviewed, this is handed over to the parents in a suitcase. They proceed to learn about its components and its correct placement. The speech processor will be located in the pinna, and she will carry a special battery holder designed to be attached to the cloth in order to prevent the external part of the implant bearing all the weight on the retro-auricular region in young children. They get an appointment to start the post implant treatment next day.

7.POST-IMPLANT TREATMENT

After the first connection is done, the family will go to aural habilitation four times per week, 45 five minutes per session.

The first objective aims to create a sound awareness. The family receives instructions about how to register the sounds produced at home and also how to highlight the silences. The sound detection is worked over the sessions, trying to achieve an active response to the sound by the girl, in this case putting objects in a bucket.

During the first phase of the treatment, the correct operation of the device is checked at the beginning of each session, verifying that the transmitter is sending the processed sound signals to the internal device correctly.

After three months, the girl is able to detect the presence of sounds with different frequency characteristics and she even starts turning around when someone is calling her. This response will be made automatically two months later.

The girl works also the sound discrimination, making a distinction between intensity, duration, sound frequencies, etc, and she progresses over the time.

The speech sounds are shown during the treatment sessions with the objective of achieving an auditory habilitation through her implant which will create an auditory memory of the speech sounds. The girl starts imitating vowel sounds and se pronounce her first words after seven month of the implantation (words such as “mum, dad, water, bear, etc). There are also mistakes in the articulation of some words but these are not corrected due to the fact that they are considered as normal in the language acquisition process.

The interaction between the girl and her parents increases with every session. They start playing with concepts with interactive games about home objects, dolls, animals, etc. There are also several reprogramming of the device to improve the quality of the audio signal perceived by the girl and, therefore, to progress with the auditory habilitation.

The girl will learn first how to identify the sounds and subsequently how to recognize each of the words that the understands in closed sequences of two or three preselected stimuli.

Although the girl started with a bimodal language, nowadays they have stopped using the sign language and the family has started talking to her in French, mother tongue of one of the parents, and even though she still does not speak, she is able to understand it.